This document provides an overview of a pediatric hematology oncology board review presentation covering several topics:
- Pediatric hematology topics include febrile neutropenia, bleeding disorders, treatment of thalassemia and iron overload, sickle cell disease, thrombocytopenia, and anemia in children.
- Pediatric oncology topics include leukemia and lymphomas, solid tumors, and oncology emergencies such as tumor lysis syndrome, superior vena cava syndrome, and mediastinal mass.
- The document also provides example questions that would be discussed during the board review covering topics like febrile neutropenia, hematologic manifestations of COVID-19, diagnoses of anemia
Recognition & management of bradycardia pediatrics AGAkshay Golwalkar
This document provides an overview of bradycardia in children, including definitions, types, recognition, ECG characteristics, and management. It describes bradycardia as an abnormally slow heart rate for a child's age that can rapidly lead to cardiopulmonary compromise. The document outlines initial steps to stabilize a child with bradycardia, including checking for hypotension, decreased consciousness, shock, or respiratory distress. It also provides guidelines for determining the heart rate from an ECG and classifying different types of bradycardic rhythms like sinus bradycardia or heart block. Reversible causes of bradycardia are listed as well as an algorithm for treating symptomatic bradycardia with medications.
Seminar on pulmonary hemorrhage in newborn by Dr. Habib, Dr. AshfaqDr. Habibur Rahim
Pulmonary hemorrhage is bleeding into the lungs that commonly affects premature infants. It presents with bloody secretions from the endotracheal tube and causes rapid clinical deterioration. Risk factors include prematurity, respiratory problems, sepsis, and mechanical ventilation. The pathophysiology involves stress on capillaries from transmural pressure, alveolar surface tension, and lung inflation. Management focuses on supportive care like ventilation, volume expansion, and transfusions to improve oxygenation and stop the bleeding. Prognosis depends on the severity and underlying causes, with mortality up to 50% in very premature infants.
This document provides details on the case of an 8 month old female child named Azma who presented with recurrent cold, cough, loose motions, and rapid breathing. It summarizes her birth history, developmental milestones, family history, clinical features, genetic causes, common health issues, diagnostic criteria, management, and prognosis of Down syndrome.
This document summarizes the history and mechanisms of jaundice and kernicterus. Some key points include:
- Kernicterus was first described in 1875 and results from bilirubin accumulation in the brain due to hyperbilirubinemia.
- Bilirubin exists in two forms - a water-soluble dianion and insoluble bilirubin acid. The acid form can cross the blood brain barrier.
- Two transporters, MRPs and MDR/PGP, help prevent bilirubin entry into the brain from the blood under normal conditions.
- Kernicterus presents as acute bilirubin encephalopathy with symptoms ranging
An 8 year old female presented with signs of septic shock including a heart rate of 180, respiratory rate of 35, and hypotension. Initial assessments found a temperature of 39.9°F, respiratory rate of 32 breaths/min, blood pressure of 70/50 mmHg, and oxygen saturation of 90% on room air. The patient appeared tired and had delayed capillary refill of 4 seconds.
Paediatric septic shock remains a significant cause of morbidity and mortality worldwide. Early goal directed therapy is crucial and aims to achieve specific clinical targets within 6 hours such as a central venous pressure of 8-12 mmHg, mean arterial pressure over 65 mmHg, urine output over 0.5 ml/kg/
This document discusses persistent pulmonary hypertension of the newborn (PPHN) with a focus on management in resource-limited settings. It provides background on PPHN, including associated conditions, signs and symptoms, diagnostic testing, and supportive care strategies. Key interventions discussed include inhaled nitric oxide (iNO), high frequency ventilation (HFV), and sildenafil. While iNO and HFV are standard treatments, their high costs limit use in many resource-poor areas. The document explores using less expensive options like sildenafil and discusses how HFV could potentially be utilized more in Nepal with appropriate equipment, training, and support.
The document provides information on ascites in children, including causes, pathophysiology, clinical presentation, investigations, and management. The most common causes of ascites in children are hepatic and renal disease, though it can also be caused by cardiac disease, trauma, infection, or neoplasia. Diagnostic evaluation involves physical exam, imaging like ultrasound or CT scan, and paracentesis with ascitic fluid analysis. Management depends on the underlying cause but may include diuretics, salt restriction, liver support therapies, or treatment of the primary disease. Complications can include respiratory distress, hernias, infections like spontaneous bacterial peritonitis.
This document discusses neuroregression in children. It begins by outlining key points about neurometabolic disorders, including that they cause diverse neurological manifestations and require a systematic clinical, biochemical and imaging approach for diagnosis. It then discusses various inborn errors of metabolism classified by pathway and organelle. Clinical features of different conditions are provided, along with details about common neonatal and childhood presentations of neuroregression. The challenges in diagnosis and important clues are reviewed. Investigations and the objectives of evaluation are described. Broad management approaches and considerations for specific conditions like Hurler disease and Niemann-Pick disease type A are highlighted.
Recognition & management of bradycardia pediatrics AGAkshay Golwalkar
This document provides an overview of bradycardia in children, including definitions, types, recognition, ECG characteristics, and management. It describes bradycardia as an abnormally slow heart rate for a child's age that can rapidly lead to cardiopulmonary compromise. The document outlines initial steps to stabilize a child with bradycardia, including checking for hypotension, decreased consciousness, shock, or respiratory distress. It also provides guidelines for determining the heart rate from an ECG and classifying different types of bradycardic rhythms like sinus bradycardia or heart block. Reversible causes of bradycardia are listed as well as an algorithm for treating symptomatic bradycardia with medications.
Seminar on pulmonary hemorrhage in newborn by Dr. Habib, Dr. AshfaqDr. Habibur Rahim
Pulmonary hemorrhage is bleeding into the lungs that commonly affects premature infants. It presents with bloody secretions from the endotracheal tube and causes rapid clinical deterioration. Risk factors include prematurity, respiratory problems, sepsis, and mechanical ventilation. The pathophysiology involves stress on capillaries from transmural pressure, alveolar surface tension, and lung inflation. Management focuses on supportive care like ventilation, volume expansion, and transfusions to improve oxygenation and stop the bleeding. Prognosis depends on the severity and underlying causes, with mortality up to 50% in very premature infants.
This document provides details on the case of an 8 month old female child named Azma who presented with recurrent cold, cough, loose motions, and rapid breathing. It summarizes her birth history, developmental milestones, family history, clinical features, genetic causes, common health issues, diagnostic criteria, management, and prognosis of Down syndrome.
This document summarizes the history and mechanisms of jaundice and kernicterus. Some key points include:
- Kernicterus was first described in 1875 and results from bilirubin accumulation in the brain due to hyperbilirubinemia.
- Bilirubin exists in two forms - a water-soluble dianion and insoluble bilirubin acid. The acid form can cross the blood brain barrier.
- Two transporters, MRPs and MDR/PGP, help prevent bilirubin entry into the brain from the blood under normal conditions.
- Kernicterus presents as acute bilirubin encephalopathy with symptoms ranging
An 8 year old female presented with signs of septic shock including a heart rate of 180, respiratory rate of 35, and hypotension. Initial assessments found a temperature of 39.9°F, respiratory rate of 32 breaths/min, blood pressure of 70/50 mmHg, and oxygen saturation of 90% on room air. The patient appeared tired and had delayed capillary refill of 4 seconds.
Paediatric septic shock remains a significant cause of morbidity and mortality worldwide. Early goal directed therapy is crucial and aims to achieve specific clinical targets within 6 hours such as a central venous pressure of 8-12 mmHg, mean arterial pressure over 65 mmHg, urine output over 0.5 ml/kg/
This document discusses persistent pulmonary hypertension of the newborn (PPHN) with a focus on management in resource-limited settings. It provides background on PPHN, including associated conditions, signs and symptoms, diagnostic testing, and supportive care strategies. Key interventions discussed include inhaled nitric oxide (iNO), high frequency ventilation (HFV), and sildenafil. While iNO and HFV are standard treatments, their high costs limit use in many resource-poor areas. The document explores using less expensive options like sildenafil and discusses how HFV could potentially be utilized more in Nepal with appropriate equipment, training, and support.
The document provides information on ascites in children, including causes, pathophysiology, clinical presentation, investigations, and management. The most common causes of ascites in children are hepatic and renal disease, though it can also be caused by cardiac disease, trauma, infection, or neoplasia. Diagnostic evaluation involves physical exam, imaging like ultrasound or CT scan, and paracentesis with ascitic fluid analysis. Management depends on the underlying cause but may include diuretics, salt restriction, liver support therapies, or treatment of the primary disease. Complications can include respiratory distress, hernias, infections like spontaneous bacterial peritonitis.
This document discusses neuroregression in children. It begins by outlining key points about neurometabolic disorders, including that they cause diverse neurological manifestations and require a systematic clinical, biochemical and imaging approach for diagnosis. It then discusses various inborn errors of metabolism classified by pathway and organelle. Clinical features of different conditions are provided, along with details about common neonatal and childhood presentations of neuroregression. The challenges in diagnosis and important clues are reviewed. Investigations and the objectives of evaluation are described. Broad management approaches and considerations for specific conditions like Hurler disease and Niemann-Pick disease type A are highlighted.
This document discusses neonatal hypertension. It begins by outlining topics to be covered, including defining neonatal hypertension, measuring blood pressure in neonates, evaluating causes of hypertension, and managing hypertension. The document then focuses on questions about properly measuring blood pressure in neonates and common causes of neonatal hypertension such as renal issues. Evaluation and treatment of neonatal hypertension is also discussed, including initial testing, choosing antihypertensive medications, and considering long term outcomes. Blood pressure measurement techniques and normal ranges are emphasized.
This document provides information on evaluating hypotonia in infants. It defines muscle tone and the differences between hypotonia and weakness. Hypotonia can have central or peripheral causes. The differential diagnosis for a floppy infant is extensive and includes central conditions like genetic syndromes or brain insults and peripheral conditions involving the motor unit, nerve, neuromuscular junction or muscle. A thorough evaluation includes the infant's history, development, family history, examination and potentially genetic or metabolic testing to determine the underlying cause.
Neonatal lupus erythematosus (NLE) occurs in infants born to mothers with autoantibodies against Ro/SSA, La/SSB, or U1-RNP. It can cause cutaneous, cardiac, hepatic, and other systemic abnormalities. Maternal autoantibodies cross the placenta and are believed to damage developing tissues. The most common presentation is a transient, nonscarring rash resembling subacute cutaneous lupus erythematosus, often appearing on the face with a "raccoon eye" appearance. Cardiac involvement includes conduction abnormalities that can lead to heart failure. The condition is usually self-limited but sometimes associated with serious sequelae
Neonatal polycythemia is defined as a venous hematocrit greater than 65%. It can be normovolemic, hypervolemic, or hypovolemic. Common causes include placental transfusion, maternal conditions like diabetes, and intrauterine hypoxia. Symptoms include poor feeding, lethargy, and cardiac or neurological issues. Screening is recommended for small or large infants and those with risk factors. Management may involve partial exchange transfusion to lower the hematocrit if symptoms are present or it is over 70% without symptoms. Studies found partial exchange transfusion effectively reduces hematocrit but did not find clinical benefits in asymptomatic infants.
This document discusses bronchopulmonary dysplasia (BPD), a chronic lung disease that occurs in premature infants requiring respiratory support. It covers the definition, risk factors, pathogenesis, clinical features, prevention, and treatment of BPD. The definition has evolved over time from relying solely on oxygen need at 28 days to incorporating factors like oxygen need, pressure support, and gestational age. BPD results from lung injury and disrupted lung development due to prematurity and respiratory support. Management aims to protect the lung from injury through gentle ventilation, optimal oxygen levels, and other strategies.
Hemolytic Uremic Syndrome Induced AKI (From Pathogenesis to Bedside) - Dr. GawadNephroTube - Dr.Gawad
Thrombotic microangiopathy (TMA) refers to intraluminal platelet thrombosis in small blood vessels. TMA can be caused by conditions such as HUS, TTP, HIV, and malignant hypertension. In hemolytic uremic syndrome (HUS), TMA is caused by Shiga toxin from E. coli or other bacteria, which activates the alternative complement pathway and causes endothelial damage. The diagnostic approach to HUS and TMA involves excluding drugs, autoimmune hemolytic anemia, and other systemic diseases as causes before determining if the presentation matches Shiga toxin-HUS, atypical HUS, or TTP. Treatment of Shiga toxin-associated HUS is generally supportive with intravenous
The heart is a muscular pump made up of four chambers that drives blood through the cardiovascular system. It contains four valves that regulate blood flow between the chambers. The heart pumps blood through two main circuits - systemic circulation and pulmonary circulation. Cardiac output, the volume of blood pumped by the heart, depends on stroke volume and heart rate. Factors like preload, afterload and contractility can affect stroke volume and therefore cardiac output. Heart failure occurs when the heart cannot pump enough blood to meet the body's needs. Its causes, signs, treatment and classifications are described.
Persistent pulmonary hypertension of newborn PPHNChandan Gowda
Persistent pulmonary hypertension of the newborn (PPHN) results from failure of the normal decrease in pulmonary vascular resistance after birth, causing right-to-left shunting of blood and hypoxemia. It can be caused by underdevelopment, maldevelopment, or maladaptation of the pulmonary vasculature. Clinical features include cyanosis and respiratory distress within the first 24 hours of life. Diagnosis involves echocardiography demonstrating elevated pulmonary pressures and responding poorly to oxygen challenges. Treatment aims to reduce PVR through ventilation strategies, medications, and potentially extracorporeal membrane oxygenation.
Here are the key points from the question:
- 14-year-old girl presented with 3 weeks of high grade fever, progressive breathlessness, swelling of feet and abdomen
- No associated symptoms like chills, rigors, dysuria, coryza, jaundice or alteration in bowel habits
- Had a generalized seizure today
- Clinically pale, oral ulcers, arthritis of both knees, left sided pleural effusion, distant heart sounds, liver palpable 4cm below costal margin, free fluid
My diagnosis would be Rheumatic fever based on the following:
- History of preceding sore throat
- Fever for 3 weeks
- Arthritis of both knees
- Carditis
1. The document provides guidance on evaluating and diagnosing anemia in children. It outlines key signs, symptoms, and pointers that suggest a child may have anemia.
2. Laboratory tests that can help determine the severity and type of anemia include complete blood count, hematocrit, reticulocyte count, blood indices, and peripheral smear.
3. A thorough history, physical exam, and lab work are needed to assess if a child is anemic, determine the severity, and identify the potential cause and type, such as blood loss, decreased red blood cell production, or increased red blood cell destruction.
A case of a 3 month old boy with jaundice and pale stool is presented. On examination, he was icteric with hepatomegaly but no other abnormalities. Laboratory tests found direct hyperbilirubinemia. The objectives of the discussion are to understand neonatal cholestasis, evaluate cases, understand the differential diagnosis, and discuss treatment options. Neonatal cholestasis is prolonged conjugated hyperbilirubinemia beyond the first 14 days of life. Causes include extrahepatic conditions like biliary atresia or intrahepatic conditions like idiopathic neonatal hepatitis. Evaluation and management aim to identify treatable causes and prevent progression of liver disease.
1. Necrotizing enterocolitis is an acquired intestinal disease of unknown etiology that commonly affects premature infants. It involves necrosis of the intestinal tissue.
2. The greatest risk factor is prematurity, with risk inversely related to birth weight and gestational age. Other risk factors include genetic factors, indomethacin exposure, maternal cocaine use, G6PD deficiency, H2 blockers, antibiotics like co-amoxiclav, and conditions that decrease mesenteric blood flow.
3. While the exact cause is unknown, factors that may contribute to pathogenesis include genetic susceptibility, ischemic injury from hypotension, and dysregulated intestinal immune response to bacterial colonization in premature infants.
1. This case presents a 1.5 month old boy with pancytopenia, fever, and respiratory symptoms.
2. Initial workup showed normocytic anemia, leukopenia, thrombocytopenia, and low corrected reticulocyte count. Bone marrow aspiration found erythroid dysplasia and megaloblastic changes.
3. Further testing found B cell immune deficiency. The patient was eventually diagnosed with MYSM1 mutation, a rare cause of congenital sideroblastic anemia and immunodeficiency. He requires supportive care including transfusions and immunoglobulin therapy.
Respiratory distress is a common problem in newborns. This document discusses the epidemiology, clinical features, assessment, causes and management approaches for several major causes of respiratory distress in newborns, including meconium aspiration syndrome, respiratory distress syndrome, and transient tachypnea of newborn. It provides clinical guidance on evaluating and treating newborns presenting with respiratory distress.
The document provides information on approaching patients with potential neurodegenerative disorders, including two patient scenarios. It discusses:
1) Classifying neurodegenerative disorders as either gray matter or white matter diseases based on features like age of onset, head size, seizures, cognition, and exam findings.
2) The key is obtaining a thorough history and physical exam to determine if it is a neurodegenerative process and rule out other treatable conditions.
3) Common inherited and acquired neurodegenerative disorders are described based on features like onset age, neurological exam, investigations and whether they primarily affect gray or white matter.
Bronchopulmonary dysplasia updates_and_prevention dr falakhagfalakha
The document discusses bronchopulmonary dysplasia (BPD) and strategies for prevention. It notes that BPD results from disrupted alveolar development and remodeling of the airways, vasculature, and smooth muscle. Risk factors include prematurity, genetics, chorioamnionitis, and exposures associated with resuscitation and mechanical ventilation. Strategies discussed to prevent BPD include using lower oxygen concentrations during resuscitation, applying continuous positive airway pressure, and avoiding overinflation and atelectrauma through gentle ventilation techniques. Future research is still needed to develop more evidence-based prevention and treatment approaches for BPD.
Cranial ultrasonography (CUS) is a useful tool for screening and evaluating the neonatal brain. CUS can accurately detect intraventricular hemorrhage, cystic periventricular leukomalacia, and ventriculomegaly. It is recommended that all infants with a birth weight less than 1250 grams or gestational age less than 30 weeks receive routine CUS screening at 7-14 days of age and again at 36-40 weeks postmenstrual age. CUS is a non-invasive and portable technique that allows for repeated examinations without risk of radiation exposure. However, CUS has some limitations in visualizing certain deep or posterior structures of the brain.
This document discusses congenital lung abnormalities. It begins by introducing common congenital lung conditions and their increasing detection. It then covers lung embryology and classification of abnormalities. The most common anomalies involve the bronchopulmonary system, vasculature, or a combination. Imaging plays a key role in evaluating fetal chest masses and associated anomalies. Specific conditions discussed include pulmonary hypoplasia, sequestration, scimitar syndrome, and agenesis. Imaging findings for various congenital lung disorders are presented along with examples of clinical cases.
A simplified description of ascitic fluid analysis. Aim of the presentation is to give a very clear understanding about the analysis of ascities.
Presentation will help the medical residents diagnose the cause of fluid accumulation in abdomen and thus will guide to adopt the appropriate pathway to solve the issue.
Neonatal Sepsis by dr Hesham Tawakol, Consultant Neonatologist at Corniche Ho...mohamed osama hussein
This document discusses neonatal sepsis and the prevention of healthcare-associated infections. It begins with an overview of neonatal sepsis, including definitions, epidemiology, risk factors, clinical presentation, diagnosis, and treatment. Drug resistance is a growing problem. To prevent healthcare-associated infections, transmission must be reduced through proper hand hygiene and infection control practices like cleaning equipment. Compliance with hand hygiene is challenging but critical for patient safety.
A 14-year-old girl with type 1 diabetes mellitus presented with blurred vision in both eyes for 2 years. Examination found cloudiness in the left lens and an intraocular lens implanted in the right eye from previous cataract surgery. The patient had poor glycemic control with HbA1c of 8.8%. Surgery was performed to remove the cataract in the left eye. Close monitoring of glycemic control and regular ophthalmologic exams are important for managing diabetic complications like cataracts in pediatric patients.
This document discusses neonatal hypertension. It begins by outlining topics to be covered, including defining neonatal hypertension, measuring blood pressure in neonates, evaluating causes of hypertension, and managing hypertension. The document then focuses on questions about properly measuring blood pressure in neonates and common causes of neonatal hypertension such as renal issues. Evaluation and treatment of neonatal hypertension is also discussed, including initial testing, choosing antihypertensive medications, and considering long term outcomes. Blood pressure measurement techniques and normal ranges are emphasized.
This document provides information on evaluating hypotonia in infants. It defines muscle tone and the differences between hypotonia and weakness. Hypotonia can have central or peripheral causes. The differential diagnosis for a floppy infant is extensive and includes central conditions like genetic syndromes or brain insults and peripheral conditions involving the motor unit, nerve, neuromuscular junction or muscle. A thorough evaluation includes the infant's history, development, family history, examination and potentially genetic or metabolic testing to determine the underlying cause.
Neonatal lupus erythematosus (NLE) occurs in infants born to mothers with autoantibodies against Ro/SSA, La/SSB, or U1-RNP. It can cause cutaneous, cardiac, hepatic, and other systemic abnormalities. Maternal autoantibodies cross the placenta and are believed to damage developing tissues. The most common presentation is a transient, nonscarring rash resembling subacute cutaneous lupus erythematosus, often appearing on the face with a "raccoon eye" appearance. Cardiac involvement includes conduction abnormalities that can lead to heart failure. The condition is usually self-limited but sometimes associated with serious sequelae
Neonatal polycythemia is defined as a venous hematocrit greater than 65%. It can be normovolemic, hypervolemic, or hypovolemic. Common causes include placental transfusion, maternal conditions like diabetes, and intrauterine hypoxia. Symptoms include poor feeding, lethargy, and cardiac or neurological issues. Screening is recommended for small or large infants and those with risk factors. Management may involve partial exchange transfusion to lower the hematocrit if symptoms are present or it is over 70% without symptoms. Studies found partial exchange transfusion effectively reduces hematocrit but did not find clinical benefits in asymptomatic infants.
This document discusses bronchopulmonary dysplasia (BPD), a chronic lung disease that occurs in premature infants requiring respiratory support. It covers the definition, risk factors, pathogenesis, clinical features, prevention, and treatment of BPD. The definition has evolved over time from relying solely on oxygen need at 28 days to incorporating factors like oxygen need, pressure support, and gestational age. BPD results from lung injury and disrupted lung development due to prematurity and respiratory support. Management aims to protect the lung from injury through gentle ventilation, optimal oxygen levels, and other strategies.
Hemolytic Uremic Syndrome Induced AKI (From Pathogenesis to Bedside) - Dr. GawadNephroTube - Dr.Gawad
Thrombotic microangiopathy (TMA) refers to intraluminal platelet thrombosis in small blood vessels. TMA can be caused by conditions such as HUS, TTP, HIV, and malignant hypertension. In hemolytic uremic syndrome (HUS), TMA is caused by Shiga toxin from E. coli or other bacteria, which activates the alternative complement pathway and causes endothelial damage. The diagnostic approach to HUS and TMA involves excluding drugs, autoimmune hemolytic anemia, and other systemic diseases as causes before determining if the presentation matches Shiga toxin-HUS, atypical HUS, or TTP. Treatment of Shiga toxin-associated HUS is generally supportive with intravenous
The heart is a muscular pump made up of four chambers that drives blood through the cardiovascular system. It contains four valves that regulate blood flow between the chambers. The heart pumps blood through two main circuits - systemic circulation and pulmonary circulation. Cardiac output, the volume of blood pumped by the heart, depends on stroke volume and heart rate. Factors like preload, afterload and contractility can affect stroke volume and therefore cardiac output. Heart failure occurs when the heart cannot pump enough blood to meet the body's needs. Its causes, signs, treatment and classifications are described.
Persistent pulmonary hypertension of newborn PPHNChandan Gowda
Persistent pulmonary hypertension of the newborn (PPHN) results from failure of the normal decrease in pulmonary vascular resistance after birth, causing right-to-left shunting of blood and hypoxemia. It can be caused by underdevelopment, maldevelopment, or maladaptation of the pulmonary vasculature. Clinical features include cyanosis and respiratory distress within the first 24 hours of life. Diagnosis involves echocardiography demonstrating elevated pulmonary pressures and responding poorly to oxygen challenges. Treatment aims to reduce PVR through ventilation strategies, medications, and potentially extracorporeal membrane oxygenation.
Here are the key points from the question:
- 14-year-old girl presented with 3 weeks of high grade fever, progressive breathlessness, swelling of feet and abdomen
- No associated symptoms like chills, rigors, dysuria, coryza, jaundice or alteration in bowel habits
- Had a generalized seizure today
- Clinically pale, oral ulcers, arthritis of both knees, left sided pleural effusion, distant heart sounds, liver palpable 4cm below costal margin, free fluid
My diagnosis would be Rheumatic fever based on the following:
- History of preceding sore throat
- Fever for 3 weeks
- Arthritis of both knees
- Carditis
1. The document provides guidance on evaluating and diagnosing anemia in children. It outlines key signs, symptoms, and pointers that suggest a child may have anemia.
2. Laboratory tests that can help determine the severity and type of anemia include complete blood count, hematocrit, reticulocyte count, blood indices, and peripheral smear.
3. A thorough history, physical exam, and lab work are needed to assess if a child is anemic, determine the severity, and identify the potential cause and type, such as blood loss, decreased red blood cell production, or increased red blood cell destruction.
A case of a 3 month old boy with jaundice and pale stool is presented. On examination, he was icteric with hepatomegaly but no other abnormalities. Laboratory tests found direct hyperbilirubinemia. The objectives of the discussion are to understand neonatal cholestasis, evaluate cases, understand the differential diagnosis, and discuss treatment options. Neonatal cholestasis is prolonged conjugated hyperbilirubinemia beyond the first 14 days of life. Causes include extrahepatic conditions like biliary atresia or intrahepatic conditions like idiopathic neonatal hepatitis. Evaluation and management aim to identify treatable causes and prevent progression of liver disease.
1. Necrotizing enterocolitis is an acquired intestinal disease of unknown etiology that commonly affects premature infants. It involves necrosis of the intestinal tissue.
2. The greatest risk factor is prematurity, with risk inversely related to birth weight and gestational age. Other risk factors include genetic factors, indomethacin exposure, maternal cocaine use, G6PD deficiency, H2 blockers, antibiotics like co-amoxiclav, and conditions that decrease mesenteric blood flow.
3. While the exact cause is unknown, factors that may contribute to pathogenesis include genetic susceptibility, ischemic injury from hypotension, and dysregulated intestinal immune response to bacterial colonization in premature infants.
1. This case presents a 1.5 month old boy with pancytopenia, fever, and respiratory symptoms.
2. Initial workup showed normocytic anemia, leukopenia, thrombocytopenia, and low corrected reticulocyte count. Bone marrow aspiration found erythroid dysplasia and megaloblastic changes.
3. Further testing found B cell immune deficiency. The patient was eventually diagnosed with MYSM1 mutation, a rare cause of congenital sideroblastic anemia and immunodeficiency. He requires supportive care including transfusions and immunoglobulin therapy.
Respiratory distress is a common problem in newborns. This document discusses the epidemiology, clinical features, assessment, causes and management approaches for several major causes of respiratory distress in newborns, including meconium aspiration syndrome, respiratory distress syndrome, and transient tachypnea of newborn. It provides clinical guidance on evaluating and treating newborns presenting with respiratory distress.
The document provides information on approaching patients with potential neurodegenerative disorders, including two patient scenarios. It discusses:
1) Classifying neurodegenerative disorders as either gray matter or white matter diseases based on features like age of onset, head size, seizures, cognition, and exam findings.
2) The key is obtaining a thorough history and physical exam to determine if it is a neurodegenerative process and rule out other treatable conditions.
3) Common inherited and acquired neurodegenerative disorders are described based on features like onset age, neurological exam, investigations and whether they primarily affect gray or white matter.
Bronchopulmonary dysplasia updates_and_prevention dr falakhagfalakha
The document discusses bronchopulmonary dysplasia (BPD) and strategies for prevention. It notes that BPD results from disrupted alveolar development and remodeling of the airways, vasculature, and smooth muscle. Risk factors include prematurity, genetics, chorioamnionitis, and exposures associated with resuscitation and mechanical ventilation. Strategies discussed to prevent BPD include using lower oxygen concentrations during resuscitation, applying continuous positive airway pressure, and avoiding overinflation and atelectrauma through gentle ventilation techniques. Future research is still needed to develop more evidence-based prevention and treatment approaches for BPD.
Cranial ultrasonography (CUS) is a useful tool for screening and evaluating the neonatal brain. CUS can accurately detect intraventricular hemorrhage, cystic periventricular leukomalacia, and ventriculomegaly. It is recommended that all infants with a birth weight less than 1250 grams or gestational age less than 30 weeks receive routine CUS screening at 7-14 days of age and again at 36-40 weeks postmenstrual age. CUS is a non-invasive and portable technique that allows for repeated examinations without risk of radiation exposure. However, CUS has some limitations in visualizing certain deep or posterior structures of the brain.
This document discusses congenital lung abnormalities. It begins by introducing common congenital lung conditions and their increasing detection. It then covers lung embryology and classification of abnormalities. The most common anomalies involve the bronchopulmonary system, vasculature, or a combination. Imaging plays a key role in evaluating fetal chest masses and associated anomalies. Specific conditions discussed include pulmonary hypoplasia, sequestration, scimitar syndrome, and agenesis. Imaging findings for various congenital lung disorders are presented along with examples of clinical cases.
A simplified description of ascitic fluid analysis. Aim of the presentation is to give a very clear understanding about the analysis of ascities.
Presentation will help the medical residents diagnose the cause of fluid accumulation in abdomen and thus will guide to adopt the appropriate pathway to solve the issue.
Neonatal Sepsis by dr Hesham Tawakol, Consultant Neonatologist at Corniche Ho...mohamed osama hussein
This document discusses neonatal sepsis and the prevention of healthcare-associated infections. It begins with an overview of neonatal sepsis, including definitions, epidemiology, risk factors, clinical presentation, diagnosis, and treatment. Drug resistance is a growing problem. To prevent healthcare-associated infections, transmission must be reduced through proper hand hygiene and infection control practices like cleaning equipment. Compliance with hand hygiene is challenging but critical for patient safety.
A 14-year-old girl with type 1 diabetes mellitus presented with blurred vision in both eyes for 2 years. Examination found cloudiness in the left lens and an intraocular lens implanted in the right eye from previous cataract surgery. The patient had poor glycemic control with HbA1c of 8.8%. Surgery was performed to remove the cataract in the left eye. Close monitoring of glycemic control and regular ophthalmologic exams are important for managing diabetic complications like cataracts in pediatric patients.
Hemolytic disease of the newborn. Diagnosis & TreatmentEneutron
Hemolytic disease of the newborn (HDN) occurs when maternal antibodies destroy fetal red blood cells. This can cause anemia, jaundice, and in severe cases, hydrops fetalis. The document discusses the classifications and causes of HDN as well as methods for diagnosing and treating affected fetuses and newborns. Key tests include blood typing the mother and fetus, direct antiglobulin testing, and measuring bilirubin levels. Treatment may involve intrauterine transfusions for severe cases, phototherapy for mild cases, or exchange transfusions for newborns with high bilirubin levels.
This document contains 29 multiple choice questions related to medical microbiology and virology. The questions cover topics such as gram-positive bacterial cell walls, influenza virus antigenic shift, fungal and bacterial infections, antibiotic modes of action, viral hepatitis, HIV and other blood-borne pathogens, tuberculosis drug interactions, and laboratory tests for various microorganisms.
Drs. Lena, Avery, and Davis’s CMC Abdominal Imaging Mastery Project: August C...Sean M. Fox
Dr. Kelsey Lena is an Emergency Medicine Resident and Drs. Michael Avery and Joshua Davis are Surgery Residents at Carolinas Medical Center in Charlotte, NC. They are interested in medical education. With the guidance of Drs. Kyle Cunningham and Michael Gibbs, they aim to help augment our understanding of emergent abdominal imaging. Follow along with the EMGuideWire.com team as they post these monthly educational, self-guided radiology slides. This month’s topics include:
• Splenic Rupture
• Obstructive jaundice
• Ovarian Torsion
Sickle cell disease is a genetic blood disorder characterized by abnormal, rigid red blood cells that can cause painful crises and organ damage. The document discusses the clinical features, pathophysiology, epidemiology, predictors of complications, and considerations for preoperative preparation and anesthesia management in patients with sickle cell disease undergoing surgery. Managing risks like dehydration, hypoxia, acidosis and low temperature is important to prevent sickle cell crises in the perioperative period.
JR_Digestive_Kelompok 2_Acute Hepatitis of Unknown Cause.pptrenno5
This document summarizes a case series of 44 children in the UK who presented with acute hepatitis of unknown cause between January and April 2022. The majority (86%) spontaneously improved, while 6 children (14%) experienced acute liver failure requiring transplantation. Human adenovirus was detected via molecular testing in 90% of cases tested. While the exact cause remains unclear, a working hypothesis is an abnormal immune response to a common virus like adenovirus in the context of reduced exposure during pandemic lockdowns. Ongoing investigation is needed to better understand the natural history and immune mechanisms involved.
This document discusses bacterial meningitis in children over 1 month old. It covers the incidence, common causative organisms by age group, clinical features, evaluation, diagnosis and treatment. Some key points:
- The incidence is highest in children under 2 months (80.69 per 100,000) and declines with age.
- Common causative organisms vary by age but include Group B Strep, pneumococcus, meningococcus and gram-negatives.
- Clinical features depend on age but may include fever, irritability, vomiting, headache and nuchal rigidity.
- Evaluation involves blood and CSF tests including cell count, glucose, protein and cultures. Imaging is only needed if signs of
This document contains 20 self-assessment questions submitted by junior medical students on their Pediatric Clerkship rotation. The questions cover topics like rheumatic fever, Kawasaki disease, asthma severity classifications, otitis media, Down syndrome, HIV in mothers and newborns, Wilson's disease, and more. The questions are provided "as is" by the clerkship director as a study aid for subsequent students.
This document contains a knowledge check for a nursing course. It includes 14 scenarios covering various medical conditions and asks questions related to each scenario. The scenarios cover topics like acute lymphoblastic leukemia, sickle cell disease, hemophilia, myelomeningocele, patent ductus arteriosus, lead poisoning, sudden infant death syndrome, Kawasaki disease, asthma, cystic fibrosis, idiopathic scoliosis, hemolytic uremic syndrome, pituitary dwarfism, and osteogenesis imperfecta. For each scenario, students are asked 1-2 questions testing their understanding of the condition's presentation, pathophysiology, diagnosis, or management. Responses of at least 2-4 sentences in length are required.
UCMS:Final Integrated medical quiz 2018 Illuminous
The document describes the rules and format for an integrated medical quiz finals round consisting of 12 total questions divided into 2 questions per team. Teams have 1 minute to answer each question they receive before it bounces to the next team, and can pounce within 30 seconds to steal a question. Correct answers earn points while incorrect answers during a pounce result in negative points, with scores announced at the end.
The document provides the details of a quiz competition involving 12 total medical questions that will be divided among teams to answer within time limits, with opportunities to earn or lose points depending on correct or incorrect responses when pouncing to answer another team's question. S
The document provides information about nephrotic syndrome in children, including:
- A case presentation of a 2-year-old male toddler with nephrotic syndrome presenting with fever, cough, facial swelling, decreased urine output, and hypoalbuminemia.
- Definitions of nephrotic syndrome as characterized by massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. The most common cause is idiopathic nephrotic syndrome.
- Histologic classifications of nephrotic syndrome including minimal change disease, focal segmental glomerulosclerosis, mesangial proliferative glomerulonephritis, and membranoproliferative
This document summarizes a review article on Kawasaki disease published in The Pediatric Infectious Disease Journal. The review discusses Kawasaki disease as an acute multisystem vasculitic syndrome of unknown etiology that primarily affects young children. While an infectious agent is suspected, no single cause has been identified. The review examines evidence for infectious and immune-mediated etiologies. It also summarizes clinical features and complications of the disease, and discusses the role of infection, immunity, and genetics in the pathogenesis of this condition.
This document discusses infective endocarditis (IE) in pediatrics. It covers the epidemiology, pathogenesis, microbiology, diagnosis, treatment, prevention, and prognosis of IE. Some key points include:
- IE mortality has decreased from nearly 100% pre-antibiotics to 15-25% currently. Risk factors include congenital heart disease and prosthetic valves.
- Endothelial damage from trauma or infection allows platelet/fibrin deposition and bacterial colonization on heart valves, leading to IE.
- Common causative organisms are viridans streptococci, staphylococci, and HACEK bacteria. Diagnosis utilizes modified Duke criteria including blood cultures and echocardiogram
Ranitidine is associated with infections, necrotizing enterocolitisCMCH,Vellore
This study found that ranitidine use in very low birth weight newborns was associated with higher rates of infections, necrotizing enterocolitis (NEC), and mortality. Newborns treated with ranitidine had over 5 times higher risk of infections like sepsis and pneumonia compared to those not treated. Rates of NEC and mortality were also significantly higher in newborns receiving ranitidine. The study cautions against the use of ranitidine in preterm newborns due to these risks of severe infectious diseases and fatal outcomes.
Ranitidine is associated with infections, necrotizing enterocolitisCMCH,Vellore
This study found that the use of ranitidine in very low birth weight newborns was associated with higher rates of infections, necrotizing enterocolitis (NEC), longer hospital stays, and higher mortality. The study prospectively examined 274 newborns, 91 of which received ranitidine treatment. Newborns treated with ranitidine had over 5 times higher risk of infections like sepsis and pneumonia. They also had a higher risk of NEC and mortality. The results suggest ranitidine should be used cautiously in preterm infants due to these risks.
Hemolytic Disesase of Newborn to ABO incompatibiltyAmanatusSholihah5
This case report describes a newborn with hemolytic disease due to ABO incompatibility between an O blood group mother and an A blood group neonate. The neonate developed severe jaundice 12 hours after a cesarean section delivery. Testing found the neonate had blood group A while the mother was blood group O. The neonate received phototherapy treatment and recovered well. This case highlights the need to screen for antibodies to both ABO and Rh blood groups during prenatal care, as hemolytic disease from ABO incompatibility can occasionally be severe as in this case. It is important for healthcare providers and the public to be aware of hemolytic disease of the newborn.
This document discusses meningitis and provides information on various types through multiple questions and case studies. It begins by stating that meningococcal meningitis has a case fatality rate of 80% without treatment, which can be reduced to less than 10% with early diagnosis and treatment. Vaccination is recommended for adolescents. Rifampicin or meningococcal vaccine is used for chemoprophylaxis of close contacts. The case studies provide details on patients presenting with meningitis caused by Neisseria meningitidis, group B streptococcus, and enterovirus.
This case report describes a 10-year-old boy presenting with nausea, vomiting, jaundice and abdominal pain. Laboratory and histopathological studies showed features of both Wilson's disease and autoimmune hepatitis. The patient was found to have elevated liver enzymes and copper levels as well as positive autoantibodies. Liver biopsy showed interface hepatitis and high copper content. Given features of both diseases, the patient was treated with immunosuppressants, penicillamine and showed improvement. The report highlights the rare occurrence of simultaneous Wilson's disease and autoimmune hepatitis. Proper diagnosis requires consideration of both diseases and combined treatment may have superior benefits for these patients.
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2nd Pediatric On Squares Pediatric Board Review.pdf
1. Pediatric Hematology Oncology For
Pediatric Board Review
2 N D P E D I AT R I C O N S Q U A R E S B O A R D R E V I E W
6 - 1 3 A U G U S T 2 0 2 2
I B R A H I M A L - H A R B I , M D , F R C P C
C O N S U LTA N T, P E D I AT R I C H E M ATO L O G Y O N C O L O G Y
F R I D AY A U G U S T 1 2 , 2 0 2 2
Pediatric Board Review Hematology Oncology August 12, 2022
2. Objectives
“Based on Pediatric Saudi Board Objectives”
Pediatric Hematology
— Febrile neutropenia
— Bleeding disorders (Coagulation
Disorders, ITP)
— Treatment of Thalassemia
— Treatment of Thalassemia Iron Overload
— Sickle cell disease
¡ Vaso-occlusive crises
¡ Infection
¡ Acute Splenic Sequestration
¡ Aplastic crisis
— Thrombocytopenia & Platelet Disorders
— Anemia in children:
¡ Approach & Management
Pediatric Oncology
— Leukemia & lymphomas
— Solid Tumors
— Oncology Emergencies
¡ Tumor Lysis Syndrome
¡ Superior Vena Cava
Syndrome
¡ Mediastinal Mass
— Side Effects of
Chemotherapy
Pediatric Board Review Hematology Oncology August 12, 2022
4. Question 1
A 3 year old girl is not known prior to have any medical problem, presented to the emergency
department with 3 days history of runny nose, dry cough, and high fever. On examination: Temp
39.7 PO, HR 155, BP 100/55, RR 16, O2 Sats 99% on Room Air. No lymphadenopathy. No
hepatosplenomegaly. Laboratory investigations showed WBC 3.1 x 109/L , Hemoglobin 12.3 g/dl,
Platelets of 623. Absolute Neutrophils Count was 90 (0.09 x 109/L ). Blood film showed relative
lymphocytosis with reactive/atypical lymphocytes. What would be the next best action ?
A. Blood Culture, Start IV Piperacillin/Tazobactam (Tazocin) and Admit
B. Blood Culture, One Dose of Ceftriaxone, and Follow up with ER next day
C. Blood Culture, One Dose Ceftriaxone and follow up in 1 week
D. Reassure family this is a viral illness and no need for any antibiotics
E. Discharge patient from ER on a course of oral antibiotics
Pediatric Board Review Hematology Oncology August 12, 2022
5. Question 1
A 3 year old girl is not known prior to have any medical problem, presented to the emergency
department with 3 days history of runny nose, dry cough, and high fever. On examination: Temp
39.7 PO, HR 155, BP 100/55, RR 16, O2 Sats 99% on Room Air. No lymphadenopathy. No
hepatosplenomegaly. Laboratory investigations showed WBC 3.1 x 109/L , Hemoglobin 12.3 g/dl,
Platelets of 623. Absolute Neutrophils Count was 90 (0.09 x 109/L ). Blood film showed relative
lymphocytosis with reactive/atypical lymphocytes. What would be the next best action ?
A. Blood Culture, Start IV Piperacillin/Tazobactam (Tazocin) and Admit
B. Blood Culture, One Dose of Ceftriaxone, and Follow up with ER next day
C. Blood Culture, One Dose Ceftriaxone and follow up in 1 week
D. Reassure family this is a viral illness and no need for any antibiotics
E. Discharge patient from ER on a course of oral antibiotics
Pediatric Board Review Hematology Oncology August 12, 2022
6. Viral Infection Associated Neutropenia (VIAN)
— Infectious / Post infectious neutropenia
— Very common
— Two Mechanisms ??
— Diagnosis ? Empirical / Clinical
— Management ?
— Prognosis ?
Pediatric Board Review Hematology Oncology August 12, 2022
7. Neutropenia In Children
— Neutropenia in Children is an extremely common condition
— Estimated 1 in 5 children admitted suffer from Neutropenia
— ANC = (Neutrophil % + Bands %) x WBC / 100
— It frustrates a lot of pediatricians with regard what to do with it
— Most Neutropenia in children is benign, non-life threatening
— Generally divided them into 2 big etiologies: Acquired & Congenital
— This classification is clinically important, and it is relevant.
— Congenital Neutropenia, albeit rare, is a serious condition & can
result in death.
Pediatric Board Review Hematology Oncology August 12, 2022
9. Risk of Invasive Bacterial Infections in Noncancer
Febrile Neutropenia
Sung L, Johnston DL. Approach to febrile neutropenia in the general paediatric
setting. Paediatr Child Health. 2007;12(1):19-21. doi:10.1093/pch/12.1.19
Lower-Risk Conditions Higher-Risk Conditions
• Familial Benign Neutropenia
• Autoimmune Neutropenia
• Chronic Benign Neutropenia
• Viral Infection-Associated Neutropenia (VIAN)
• Severe Congenital Neutropenia (Kostmann Syndrome)
• Cyclic Ceutropenia
• Shwachman-Diamond Syndrome
• Reticular Dysgenesis
• Myelokathexis
• Aplastic anemia
Pediatric Board Review Hematology Oncology August 12, 2022
10. Factors That Increase Risk of Invasive Bacterial
Infection in Febrile Neutropenia
August 12, 2022
Pediatric Board Review Hematology Oncology
1. Mucositis
2. Intravascular devices
3. Depression of other immune function
4. Abnormal neutrophil function
5. Longer duration of neutropenia (eg, >7 days)
6. Lower absolute neutrophil count (eg, ≤100 cells/μL)
7. Concurrent immunosuppressant therapy
Sung L, Johnston DL. Approach to febrile neutropenia in the general paediatric setting. Paediatr Child
Health. 2007;12(1):19-21. doi:10.1093/pch/12.1.19
11. Management of Non-Chemotherapy Induced
Febrile Neutropenia
Monotherapy vs. Dual Drugs
Pediatric high-risk FN and as empirical therapy, use monotherapy:
Antipseudomonal Beta Lactam, or
4th Generation cephalosporin, or
Carbapenem
Reserve addition of a second gram negative agent:
1. Clinically unstable
2. When a resistant infection is suspected
3. In centers with a high rate of resistant pathogens
Robinson PD, Lehrnbecher T, Phillips R, et al: Strategies for empiric management of pediatric fever and neutropenia in patients with cancer and hematopoietic stem-cell
transplantation recipients: A systematic review of randomized trials. JCO 34:2054-2060, 2016
Pediatric Board Review Hematology Oncology August 12, 2022
12. Question 2
— A 5 year old boy came to the emergency department with high fevers. He was found to
have COVID-19 infection confirmed by PCR test. Laboratory investigations showed:
WBC 3.1 x 109/L , Hemoglobin 11.5 g/dl, Platelets of 46. Lymphocytes were 0.34 x
109/L (ALC 340). Neutrophils 1.7 x 109/L (ANC 1700). PT 17.2 (12.1-14.5), INR 1.4
(0.87-1.2), PTT 47 (33.6-402.5). D-Dimer (2-12 years old, 0.4-2.27 mg/L). What are the
most prognostically significant hematologic manifestations of COVID-19 infection in
children:
A. Lymphopenia
B. Thrombocytopenia
C. High Level D-Dimer
D. A & B
E. A & C
Pediatric Board Review Hematology Oncology August 12, 2022
13. Question 2
— A 5 year old boy came to the emergency department with high fevers. He was found to
have COVID-19 infection confirmed by PCR test. Laboratory investigations showed:
WBC 3.1 x 109/L , Hemoglobin 11.5 g/dl, Platelets of 46. Lymphocytes were 0.34 x
109/L (ALC 340). Neutrophils 1.7 x 109/L (ANC 1700). PT 17.2 (12.1-14.5), INR 1.4
(0.87-1.2), PTT 47 (33.6-402.5). D-Dimer (2-12 years old, 0.4-2.27 mg/L). What are the
most prognostically significant hematologic manifestations of COVID-19 infection in
children:
A. Lymphopenia
B. Thrombocytopenia
C. High Level D-Dimer
D. A & B
E. A & C
Pediatric Board Review Hematology Oncology August 12, 2022
14. Hematological Manifestations of SARS-CoV-2 in Children
— The majority of infected children had a normal leukocyte count
— The most common WBC abnormality was leukopenia.
— Lymphopenia, less common but may be a marker of severe disease
— Neonates & infants: the most common abnormality: lymphocytosis
— Anemia & hypercoagulability: higher in children in MIS-C with
SARS-CoV-2.
Kosmeri C, Koumpis E, Tsabouri S, Siomou E, Makis A. Hematological manifestations of SARS-CoV-2 in children. Pediatr Blood Cancer. 2020 Dec;67(12):e28745
Pediatric Board Review Hematology Oncology August 12, 2022
15. Question 3
— An 11 months old boy came to your clinic with pallor. You ordered CBC
and Hemoglobin was 6.1 g/dl, MCV 51 fL, MCH 19 pg. Hemoglobin
Electrophoresis showed Hemoglobin F 94.3%, Hemoglobin A2 5.5%.
What is the most likely diagnosis:
A. Iron Deficiency Anemia
B. Beta Thalassemia Major
C. Beta Thalassemia Minor
D. Sickle Cell Disease
E. Homozygous Hb E Disease
Pediatric Board Review Hematology Oncology August 12, 2022
16. Question 3
— An 11 months old boy came to your clinic with pallor. You ordered CBC
and Hemoglobin was 6.1 g/dl, MCV 51 fL, MCH 19 pg. Hemoglobin
Electrophoresis showed Hemoglobin F 94.3%, Hemoglobin A2 5.5%.
What is the most likely diagnosis:
A. Iron Deficiency Anemia
B. Beta Thalassemia Major
C. Beta Thalassemia Minor
D. Sickle Cell Disease
E. Homozygous Hb E Disease
Pediatric Board Review Hematology Oncology August 12, 2022
17. Beta Thalassemia Major
— Thalassemia: Greek: Thalass: Sea, Emeia: Blood
— As a disease, first recognized 1925 by Thomas Cooley & Pearl
Lee
¡ A series of infants who became profoundly anemic
¡ Splenomegaly and Bone Changes over the first year of life
— The word ”Thalassemia” was used for the 1st time in 1932
— In 1940, the genetic character of this disorder was fully appreciated
— Most patients come from the Mediterranean region
Whipple GH, Bradford WI. Am J Dis Child 1932;44:336
Pediatric Board Review Hematology Oncology August 12, 2022
18. Beta Thalassemia Major
— Thalassemia: Genetic disorders of Hemoglobin Synthesis
— Characterized by reduction in synthesis of one or more of
the Globin Chains
— This leads to:
¡ Imbalanced globin-chain synthesis
¡ Defective hemoglobin production
¡ Severe Anemia
(Victor et al., 1999)
Pediatric Board Review Hematology Oncology August 12, 2022
19. Diagnosis of Beta Thalassemia
— CBC:
¡ Increased RBC mass
¡ Hypochromic Microcytic anemia with normal RDW, Why ??
— HPLC:
¡ Beta Thalassemia Minor:
÷ Hb F 0.1-5%
÷ Hb A2 ³ 4%
¡ Beta Thalassemia Major:
÷ B0/ B0
¢ HbF up to 95%
¢ Hb A2 ³ 5%
÷ B0/B+
¢ HbF 70–90%
¢ HbA up to 30%
¢ Hb A2 ³ 4%
Pediatric Board Review Hematology Oncology August 12, 2022
20. Pediatric Board Review Hematology Oncology August 12, 2022
Question 4
— A 9 year old girl known case of Beta Thalassemia Major on regular
monthly blood transfusion presented to your clinic complaining of
fatigue, SOB, and sometimes feeling her hear beat loudly. She is on
Deferasirox but she is not compliant.
— On examination, she has tachycardia, HR 144, also tachypneic RR 54,
Os Sat 87 RA, BP 100/60. Chest X-Ray showed lung edema and
cardiomegaly. She has hepatomegaly as well. CBC showed Hemoglobin
of 10.8 g/dl (Transfused 1 week ago). Ferritin 13550 ng/ml .. The most
likely cause is:
A. Anemia
B. Congestive Heart Failure
C. Zinc Deficiency
D. Hypothyroidism
E. Vitamin D Deficiency
21. Pediatric Board Review Hematology Oncology August 12, 2022
Question 4
— A 9 year old girl known case of Beta Thalassemia Major on regular
monthly blood transfusion presented to your clinic complaining of
fatigue, SOB, and sometimes feeling her hear beat loudly. She is on
Deferasirox but she is not compliant.
— On examination, she has tachycardia, HR 144, also tachypneic RR 54,
Os Sat 87 RA, BP 100/60. Chest X-Ray showed lung edema and
cardiomegaly. She has hepatomegaly as well. CBC showed Hemoglobin
of 10.8 g/dl (Transfused 1 week ago). Ferritin 13550 ng/ml .. The most
likely cause is:
A. Anemia
B. Congestive Heart Failure
C. Zinc Deficiency
D. Hypothyroidism
E. Vitamin D Deficiency
23. Thalassaemia: Iron Overload
Hepatic fibrosis à Cirrhosis
Arrhythmia
Hypogonadism
Diabetes
Hypothyroidism
Hypoparathyroidism
Cardiomyopathy
Pediatric Board Review Hematology Oncology August 12, 2022
24. How do we know if there’s too much iron?
Pediatric Board Review Hematology Oncology August 12, 2022
— Serum ferritin concentration
¡ Used in clinical practice globally
— Liver biopsy
¡ Reference methodology (‘gold standard’)
— Magnetic resonance imaging (T2*MRI)
¡ The CMR with measurement of T2* relaxation time should be
performed in all patients with idiopathic cardiomyopathy.
— Echocardiogram
25. Cardiomyopathy & CHF in Iron Overload
Pediatric Board Review Hematology Oncology August 12, 2022
— Iron deposition in the heart often causes arrhythmias and
progressive systolic dysfunction.
— Manifests as dilated cardiomyopathy with low LVEF
— Can be reversible only if it is diagnosed and treated in
its early stages.
Rivers J, Garrahy P, Robinson W, et al. Reversible cardiac dysfunction in hemochromatosis. Am Heart J 1987; 113: 216-7.
26. Cardiomyopathy & CHF in Iron Overload
Pediatric Board Review Hematology Oncology August 12, 2022
— Patients may be asymptomatic early in the disease.
— Once heart failure develops, there is rapid deterioration.
— Cardiac Assessment is characterized by
¡ Dilated cardiomyopathy with dilated ventricles,
¡ Reduced ejection fraction
¡ Reduced fractional shortening
Rivers J, Garrahy P, Robinson W, et al. Reversible cardiac dysfunction in hemochromatosis. Am Heart J 1987; 113: 216-7.
27. Hypochromic Microcytic Anemia
Pediatric Board Review Hematology Oncology August 12, 2022
— How do you differentiate Hypochromic Microcytic
Anemia that is due to Thalassemia carrier from Iron
Deficiency Anemia only using CBC ?
¡ RBC
¡ MCV
¡ RDW
¡ Mentzer Index
28. Pediatric Board Review Hematology Oncology August 12, 2022
Question 5
A pregnant woman who is 28 year old who has sickle cell disease and
asks you what are the chances her newborn baby would have Sickle
Cell Disease. Her husband has Sickle Cell /Beta Thalassemia Disease.
Choose the BEST answer:
A. 25% chance of having Sickle cell disease
B. 50% chance of having Sickle cell disease
C. 75% chance of having Sickle Cell Disease
D. 100% chance of having Sickle cell disease
E. 50% chance of SCD, 50% chance of Sickle Cell/Beta Thalassemia
29. Pediatric Board Review Hematology Oncology August 12, 2022
Question 5
A pregnant woman who is 28 year old who has sickle cell disease and
asks you what are the chances her newborn baby would have Sickle
Cell Disease. Her husband has Sickle Cell /Beta Thalassemia Disease.
Choose the BEST answer:
A. 25% chance of having Sickle cell disease
B. 50% chance of having Sickle cell disease
C. 75% chance of having Sickle Cell Disease
D. 100% chance of having Sickle cell disease
E. 50% chance of SCD, 50% chance of Sickle Cell/Beta
Thalassemia
30. Mother
Father
S S
S SS SS
Beta S/Beta S/Beta
SCD Mother and SCD/Beta Thalassemia Father
Pediatric Board Review Hematology Oncology August 12, 2022
31. Pediatric Board Review Hematology Oncology August 12, 2022
Sickle Cell Disease’ Syndromes
— Sickle Cell Disease genotypes in order of severity
• Hb SS
• Hb S/βo
• Hb S/β+
• Hb S/α Thal
• Hb S/C
Maria Stella Figueiredo, The compound state: Hb S/beta-thalassemia Universidade Federal de São Paulo (UNIFESP), São Paulo, SP, Brazil, rev bras
hematol hemoter. 2 0 1 5;37(3):150–152
32. Pediatric Board Review Hematology Oncology August 12, 2022
Sickle Cell Syndromes in KSA
— Sickle Cell Disease genotypes in order of frequency in
KSA
• Hb SS 72%
• Hb SS with high Hb F 15%
• Hb S/Beta 8 %
• Hb S/Alpha Thal 4-6%
• Hb S/C 1-2 %
El-Hazmi MA. On the nature of sickle-cell disease in the Arabian Peninsula. Hum Genet. 1979;52:323–35.
34. Pediatric Board Review Hematology Oncology August 12, 2022
Question 6
— An 8 year old boy known case of sickle cell disease presented with an inability to walk
on his right leg. On exam, he was febrile 39 °C. He has a focal tenderness over right
distal femur. No pain in any other parts of his body. Labs showed WBC of 29.7 ×
109/L, Hemoglobin 7.3 g/dl (Usually 7-8 g/dl). Platelets of 381. CRP was 131 mg/L,
ESR 96 mm/hr. The most likely etiologic organism causing this condition would be:
A. Salmonella
B. Staphylococcus aureus
C. E. coli
D. Streptococcus group A
E. Streptococcus pneumonia
35. Pediatric Board Review Hematology Oncology August 12, 2022
Question 6
— An 8 year old boy known case of sickle cell disease presented with an inability to walk
on his right leg. On exam, he was febrile 39 °C. He has a focal tenderness over right
distal femur. No pain in any other parts of his body. Labs showed WBC of 29.7 ×
109/L, Hemoglobin 7.3 g/dl (Usually 7-8 g/dl). Platelets of 381. CRP was 131 mg/L,
ESR 96 mm/hr. The most likely etiologic organism causing this condition would be:
A. Salmonella
B. Staphylococcus aureus
C. E. coli
D. Streptococcus group A
E. Streptococcus pneumonia
36. Pediatric Board Review Hematology Oncology August 12, 2022
Osteomyelitis in Children with SCD
— The most serious bone involvement in children with SCD
— Diagnostic Dilemma:
• Clinical
÷ Fever
÷ Inflammatory markers
÷ “Focality”
÷ Decrease range of motion UL / Non-Weight bearing LL
• Radiological:
÷ U/S
÷ Bone scan
÷ MRI
• Why do we care ?
37. Pediatric Board Review Hematology Oncology August 12, 2022
Osteomyelitis in Children with SCD
— How to differentiate Osteomyelitis from Bone Infarction (VOC) in children with SCD
¡ Kocher’s Criteria. (Originally for Septic Arthritis)
÷ Fevers > 38 C
÷ ESR > 40 mm
÷ WBC > 12, 000
÷ Decreased ROM Upper Limbs / Non-Weight Bearing Lower Limbs
¡ MRI: (Gold Standard Imaging Modality))
÷ Periosteal Reaction
÷ Soft Tissue Enhancement
÷ Soft Tissue Edema
÷ Presence of Sequestrum
¡ Bone Scan: Useless, Uptake increased in VOC & Bone Infections
¡ Bone Marrow Scan: decreased uptake on bone marrow scan in a patient with SCD & bone pain almost
invariably indicates infarction, whereas normal uptake strongly suggests the diagnosis of osteomyelitis. Not
widely available
Yolande, D. , Tufong, K. , Jules, T. , Mayah, A. , Charlotte, E. , Njinkui, D. , Enyama, D. , Selangai, H. and Siysi, V. (2021) Osteomyelitis in Children with Sickle Cell Disease: A Challenging Diagnosis: Case Report from
Cameroon. Open Journal of Pediatrics, 11, 208-214.
Sreedhar Rao, MD et al, Scintigraphic differentiation of bone infarction from osteomyelitis in children with sickle cell disease. The Journal of Pediatrics, November 1985, Pages 685-68
38. Score Likelihood of septic arthritis
1 3%
2 40%
3 93%
4 99%
The Kocher criteria are a tool useful in the differentiation of septic
arthritis from transient synovitis in the child with a painful hip.
They are named for Mininder S. Kocher
Pediatric Board Review Hematology Oncology August 12, 2022
39. Pediatric Board Review Hematology Oncology August 12, 2022
Question 7
A 5 year old boy known case of SCD presented to the emergency department
complainging of severe pain in his lower legs. No fevers and no other complaints.
Labs showed WBC 7.1 × 109/L, Hb 8.3 g/dl (his baseline). Platelets 345 × 109/L ,
CRP 35 mg/ml. Pain Score was 9/10. He required one IV Morphine bolus 0.1
mg/kg IV. Pain improved temporarily. After 2 hrs, severe pain returns, and he is
crying in pain. Pain now is 10/10. The best next plan of action:
A.Give a second Morphine bolus 0.1 mg/kg IV and start IV Morphine infusion
B.Give a 2nd Morphine bolus at 0.05 mg/kg and start IV Morphine infusion
C.He is faking it since he took a dose of Morphine 2 hrs ago, so nothing else
D.Give Paracetamol 15 mg/kg PO and observe
E. Give Paracetamol 15 mg/kg IV and observe
40. Pediatric Board Review Hematology Oncology August 12, 2022
Question 7
A 5 year old boy known case of SCD presented to the emergency department
complainging of severe pain in his lower legs. No fevers and no other complaints.
Labs showed WBC 7.1 × 109/L, Hb 8.3 g/dl (his baseline). Platelets 345 × 109/L ,
CRP 35 mg/ml. Pain Score was 9/10. He required one IV Morphine bolus 0.1
mg/kg IV. Pain improved temporarily. After 2 hrs, severe pain returns, and he is
crying in pain. Pain now is 10/10. The best next plan of action:
A.Give a second Morphine bolus 0.1 mg/kg IV and start IV Morphine infusion
B.Give a 2nd Morphine bolus at 0.05 mg/kg and start IV Morphine infusion
C.He is faking it since he took a dose of Morphine 2 hrs ago, so nothing else
D.Give Paracetamol 15 mg/kg PO and observe
E. Give Paracetamol 15 mg/kg IV and observe
42. Pediatric Board Review Hematology Oncology August 12, 2022
Vaso-Occlusive Crisis
— A VOC is the hallmark acute complication of SCD
— Manifests as severe acute pain
— Although VOCs are typically associated with excruciating pain
of sudden onset, some people experience gradual onset of a
VOC
— All SCD will experience a VOC at least once during their
lifetime
— About 50% of VOC in Children with SCD is Unprovoked !
43. Pediatric Board Review Hematology Oncology August 12, 2022
How To Manage Vaso-Occlusive Crisis in Children with SCD
— IV Fluids: If euvolemic, give only maintenance
— Monitor for excessive sedation by measuring sedation with an
objective measurement sedation scale and oxygenation levels.
— Gradually titrate down parenteral opioids as VOC resolves
— NO blood transfusion for VOC unless there are other indications
— In children VOC with an O2 <95 % on RA, ADMINISTER O2
— If IV Morphine is maximized
— PCA then Use Incentive Spirometer
Udezue E, Herrera E. Pain management in adult acute sickle cell pain crisis: a viewpoint. West Afr J Med. 2007;26(3):179-82
44. Pediatric Board Review Hematology Oncology August 12, 2022
Question 8
— An 8 months old girl brought by her parents because of continuous crying and swelling
in her hands for 3 days. She is not known to have any medical problems. She was
product of uncomplicated pregnancy. She was born at a peripheral hospital and
incomplete neonatal screen was done.
— Afebrile but tachycardiac. She looks in pain. Her hands were swollen and looked as in
the photo. Labs showed WBC 19.7 × 109/L, Hb 9.3 g/dl. Platelets 533 × 109/L , CRP 3
mg/ml. ESR 6 mm/hr.
— What is the most likely diagnosis ?
A. Dactylitis (Hand Foot Disease)
B. Psoriatic Arthritis
C. Fracture / Trauma
D. Child Abuse
E. Osteomyelitis
46. Pediatric Board Review Hematology Oncology August 12, 2022
Question 8
— An 8 months old girl brought by her parents because of continuous crying and swelling
in her hands for 3 days. She is not known to have any medical problems. She was
product of uncomplicated pregnancy. She was born at a peripheral hospital and
incomplete neonatal screen was done.
— Afebrile but tachycardiac. She looks in pain. Her hands were swollen and looked as in
the photo. Labs showed WBC 19.7 × 109/L, Hb 9.3 g/dl. Platelets 533 × 109/L , CRP 3
mg/ml. ESR 6 mm/hr.
— What is the most likely diagnosis ?
A. Dactylitis (Hand Foot Disease)
B. Psoriatic Arthritis
C. Fracture / Trauma
D. Child Abuse
E. Osteomyelitis
47. Pediatric Board Review Hematology Oncology August 12, 2022
Hand Foot Syndrome (Dactylitis)
— The earliest clinical musculoskeletal manifestation of SCD
— Affects infants and young children 6 months to 6 years
— Highest incidence during the first 6-12 months of life
— Infarction at metacarpals, metatarsals, and phalanges
— Results from sickling of RBCs in the capillary beds
— Histologically ??
— Risk factor for ??
48. Pediatric Board Review Hematology Oncology August 12, 2022
Question 9
— You are on call, and a nurse calls you saying the 11 year old boy known case
of SCD, who was admitted 2 days earlier with severe VOC and now on
Morphine Infusion 40 mcg/kg/hr, is developing shortness of breath and chest
pain. You come to assess him. You find him lethargic, toxic looking,
tachycardiac (130), O2 Sats 82%, and in severe respiratory distress. You assess
him and put him on 10 L. O2. You call PICU. . You order stat Chest Xray,
ABG, and blood culture and CBC LFT, RFT. CBC showed Hb 6.8 g/dl, WBC
34.6, Platelets 110. Retic Count 14%. T-Bilirubin 72 umol/L. LDH 651 IU/L..
CRP 344 mg/ml. PH 7.22, PaO2 44, PaCO2 61. Chest X-Ray showed Right
lower lobe infiltration. The best predictors of poor outcome are:
A. Chest pain, Hypoxemia, Degree of Respiratory Acidosis
B. Lobar involvement, Relative Thrombocytopenia, Hx of Cardiac Disease
C. Lobar Involvement, Hypoxemia, Level of CRP
D. Severity of VOC, Leukocytosis, CRP level
E. CRP Level, Hypoxemia, Chest pain
49. Pediatric Board Review Hematology Oncology August 12, 2022
Question 9
— You are on call, and a nurse calls you saying the 11 year old boy known case of
SCD, who was admitted 2 days earlier with severe VOC and now on Morphine
Infusion 40 mcg/kg/hr, is developing shortness of breath and chest pain. You come
to assess him. You find him lethargic, toxic looking, tachycardiac (130), O2 Sats
82%, and in severe respiratory distress. You assess him and put him on 10 L. O2.
You call PICU. . You order stat Chest Xray, ABG, and blood culture and CBC LFT,
RFT. CBC showed Hb 6.8 g/dl, WBC 34.6, Platelets 110. Retic Count 14%. T-
Bilirubin 72 umol/L. LDH 651 IU/L.. CRP 344 mg/ml. PH 7.22, PaO2 44, PaCO2
61. Chest X-Ray showed Right lower lobe infiltration. The best predictors of poor
outcome are:
A. Chest pain, Hypoxemia, Degree of Respiratory Acidosis
B. Lobar involvement, Relative Thrombocytopenia, Hx of Cardiac Disease
C. Lobar Involvement, Hypoxemia, Level of CRP
D. Severity of VOC, Leukocytosis, CRP level
E. CRP Level, Hypoxemia, Chest pain
50. Pediatric Board Review Hematology Oncology August 12, 2022
Acute Chest Syndrome
— 2nd most common cause of hospitalization
— leading cause of SCD mortality in adults
— Management is largely determined by the experience of
individual practitioners
— No conclusive randomized controlled clinical trials to guide
therapy
— 50% of ACS happen in SCD children admitted with VOC
51. Pediatric Board Review Hematology Oncology August 12, 2022
Definition of ACS
— ACS: acute pulmonary illness that occurs in patients with SCD
— ACS is currently defined as a new infiltrate on chest radiograph in conjunction with 1
other new symptom or sign:
¡ Chest pain
¡ Cough
¡ Wheezing
¡ Tachypnea
¡ Fever (> 38.5°C)
Charache S, Scott JC, Charache P. “Acute chest syndrome” in sickle cell anemia. Microbiology, treatment, and prevention. Arch Intern Med. 1979;139(1):67-69
52. Pediatric Board Review Hematology Oncology August 12, 2022
Pulmonary Fat Embolism (PFE)
— Why ACS is different than pneumonia ?
— Fat from bone marrow embolizes to the lungs
• PFE is associated with activation of:
• Secretory phospholipase A2 (sPLA2)
• Free fatty acids (Toxic to Lung Tissues)
• Serum levels of sPLA2: initially uniformly elevated in ACS
• In NACSS, 80% of subjects with ACS had sPLA2 elevation.
• The administration of PRBCs in pts with VOC & é sPLA2 leads to ?
Elliott P. Vichinsky, M.D, et al, Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease (NACSS) , N Engl J Med 2000; 342:1855-1865
53. Pediatric Board Review Hematology Oncology August 12, 2022
Clinical and Laboratory Predictors of Development of
Acute Chest Syndrome
1. Young age ?
2. Low HbF
3. High steady-state WBC
4. Severe genotypes (HbSS and HbSb0 thalassemia)
5. More than > 3 severe VOC episodes in the preceding year
6. Asthma/airway hyperreactivity
7. Tobacco smoke exposure (TSE)
8. Recent surgery
Jain S, Bakshi N, Krishnamurti L. Acute Chest Syndrome in Children with Sickle Cell Disease. Pediatr Allergy Immunol Pulmonol. 2017 Dec 1;30(4):191-201.
54. Pediatric Board Review Hematology Oncology August 12, 2022
Question 10
— A 1 year old boy known case of SCD & G6PD presented to
ER with history of runny nose and mild cough, and then
sudden onset of significant pallor. On Exam: He had mild
URTIs symptoms, T 37.1 Ax, HR 160, RR 28, BP 90/45.
— No HSM. CBC Hb 3.2 g/dl, Hct 9.6, Retic count 1.2%, LDH
420 IU/L, T-Bilirubin 42 μmol/L.
— What is the most likely diagnosis:
A.Acute Hemolytic Crisis
B.Hyperhemolytic Crisis
C.Aplastic Crisis
D.Transient Erythroblastopenia of Childhood (TEC)
E. Acute Splenic Sequestration
55. Pediatric Board Review Hematology Oncology August 12, 2022
Question 10
— A 1 year old boy known case of SCD & G6PD presented to
ER with history of runny nose and mild cough, and then
sudden onset of significant pallor. On Exam: He had mild
URTIs symptoms, T 37.1 Ax, HR 160, RR 28, BP 90/45.
— No HSM. CBC Hb 3.2 g/dl, Hct 9.6, Retic count 1.2%, LDH
420 IU/L, T-Bilirubin 42 μmol/L.
— What is the most likely diagnosis:
A.Acute Hemolytic Crisis
B.Hyperhemolytic Crisis
C.Aplastic Crisis
D.Transient Erythroblastopenia of Childhood (TEC)
E. Acute Splenic Sequestration
58. Pediatric Board Review Hematology Oncology August 12, 2022
Aplastic Crisis
— Bone marrow temporarily shuts down.
— Acute fall in haemoglobin levels - by up to 1 g per dl per day
— There is a complete absence of reticulocytes in the blood film.
— The causative agent is usually human parvovirus B19.
— This leads to profound anaemia over a period of a few days.
Koch WC, Massey GV. Aplastic crisis. Pediatr Rev. 1990 Nov;12(5):142-8
59. Pediatric Board Review Hematology Oncology August 12, 2022
Aplastic Crisis
— This virus infects RBC progenitors in bone marrow,
— In SCD, RBC lifespan is greatly shortened (usually 10-20 days),
— Very rapid drop in Hb occurs.
— Bone marrow recovery occurring in 7-10 days
— Followed by brisk Reticulocytosis
Koch WC, Massey GV. Aplastic crisis. Pediatr Rev. 1990 Nov;12(5):142-8
60. Aplastic Crisis
Pediatric Board Review Hematology Oncology August 12, 2022
— Aplastic crises is characterised by:
¡ Reticulocytopaenia – seen 5 days post exposure, last for 7–10 d
¡ Symptomatic anaemia
¡ Serum IgM antibodies to parvovirus B19 or Positive PCR
Koch WC, Massey GV. Aplastic crisis. Pediatr Rev. 1990 Nov;12(5):142-8
61. Pediatric Board Review Hematology Oncology August 12, 2022
Question 11
— You are on call, and a 2 year old boy known case of SCD,
presented to ER with pallor and inability to move his right
side. He is tachycardiac (170), but afebrile. Spleen is 6 cm
BCM (usually not-palpable). CBC showed Hb 2.1 g/dl,
WBC 6.3, Platelets 150. Retic Count 28%. T-Bilirubin 72
umol/L. LDH 432 IU/L.. MRI showed Left MCA Stroke.
The most likely diagnosis explaining the whole scenario is:
A.Acute Stroke
B.Acute Splenic Sequestration
C.Acute Hemolytic Crisis
D.Hypersplenism
E. Splenic Infarction
62. Pediatric Board Review Hematology Oncology August 12, 2022
Question 11
— You are on call, and a 2 year old boy known case of SCD,
presented to ER with pallor and inability to move his right
side. He is tachycardiac (170), but afebrile. Spleen is 6 cm
BCM (usually not-palpable). CBC showed Hb 2.1 g/dl,
WBC 6.3, Platelets 150. Retic Count 28%. T-Bilirubin 72
umol/L. LDH 432 IU/L.. MRI showed Left MCA Stroke.
The most likely diagnosis explaining the whole scenario is:
A.Acute Stroke
B.Acute Splenic Sequestration
C.Acute Hemolytic Crisis
D.Hypersplenism
E. Splenic Infarction
64. Pediatric Board Review Hematology Oncology August 12, 2022
Splenic Complications of SCD
— Acute Splenic Sequestrations
— Hypersplenism
— Splenic Infarction
— Splenic Abscess
— Splenomegaly without Sequestration
65. Pediatric Board Review Hematology Oncology August 12, 2022
Question 12
— A 1 year old boy with sudden onset of pallor, jaundice, associated
with viral illness. On examination, he has splenomegaly (6 cm
below costal margin). CBC showed Hemoglobin of 5.8 g/dl.
MCV was 80 fL, MCH was 28 pg (NR 23-31), and MCHC was
41 g/dl (NR 32-36). Reticulocyte count was 9.3 %. Platelet count
255. Direct antilobulin’s test (Coomb’s test) was negative.
Ferritin 55 ng/ml . Coagulation profile was normal. The most
likely cause is:
A. Iron deficiency anemia
B. Beta Thalassemia Major
C. Hereditary Spherocytosis
D. Pyruvate Kinase Deficiency
66. Pediatric Board Review Hematology Oncology August 12, 2022
Question 12
— A 1 year old boy with sudden onset of pallor, jaundice, associated
with viral illness. On examination, he has splenomegaly (6 cm
below costal margin). CBC showed Hemoglobin of 5.8 g/dl.
MCV was 80 fL, MCH was 28 pg (NR 23-31), and MCHC was
41 g/dl (NR 32-36). Reticulocyte count was 9.3 %. Platelet count
255. Direct antilobulin’s test (Coomb’s test) was negative.
Ferritin 55 ng/ml . Coagulation profile was normal. The most
likely cause is:
A. Iron deficiency anemia
B. Beta Thalassemia Major
C. Hereditary Spherocytosis
D. Pyruvate Kinase Deficiency
67. Hereditary Spherocytosis
Pediatric Board Review Hematology Oncology August 12, 2022
— HS is the most common RBC membranopathy (RBC Membrane Disorders)
— Autosomal Dominant Inheritance
— Characterized by anemia, jaundice, and splenomegaly, susceptibility to GB Stones
— Prevalence ??
— Clinical severity is variable
— Genetic: mutations in the 7 genes coding for RBC membranes:
¡ α spectrin
¡ β spectrin
¡ ankyrin
¡ Band 3 Deficiency
¡ Protein 4.1 Mutation
¡ Protein 4.2 mutation
¡ Actin
— Diagnosis:
¡ Blood film: ³ 15-20 % spherocytosis
¡ Osmotic Fragility Test
¡ Flowcytometry
¡ Genetic: WES/NGS
Silverio Perrotta, Patrick G Gallagher, Narla Mohandas , Hereditary spherocytosis Lancet 2008; 372: 1411–26
68. Hereditary Spherocytosis
Pediatric Board Review Hematology Oncology August 12, 2022
— The classic laboratory features of HS include the following
¡ Mild to moderate anemia
¡ Reticulocytosis
¡ Increased mean MCHC
¡ Spherocytes on the peripheral blood smear
¡ Hyperbilirubinemia
¡ Abnormal results on the incubated osmotic fragility test
— Complications of spherocytosis may include
¡ Megaloblastic / Aplastic crisis
¡ Gallbladder Stones (Cholelithiasis / Cholecystitis)
¡ Hemolytic Crisis
Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ. Guidelines for the diagnosis and management of hereditary spherocytosis - 2011 update. Br J Haematol. 2012 Jan. 156(1):37-49
69. Pediatric Board Review Hematology Oncology August 12, 2022
Question 13
— An 8 year old boy presented to the emergency department with
history of pallor and petechiae over last 4 weeks. He also has
fevers during the same period. Vague history of URTI 2 months
prior with cough, runny nose and sore throat. Examination
showed a pale child, no hepatosplenomegaly. CBC: WBC 1.1 ×
109/L, Hb 5.2 g/dl, Platelets 7, ANC 120 (0.12 × 109/L). Bone
marrow Aspiration & Biopsy was done later and showed 75 %
hypocellular marrow. The most likely diagnosis:
A. Bone Marrow Suppression post viral infection
B. Diamond Blackfan Anemia
C. Fanconi Anemia
D. Severe Aplastic Anemia
E. Very severe Aplastic Anemia
70. Pediatric Board Review Hematology Oncology August 12, 2022
Question 13
— An 8 year old boy presented to the emergency department with
history of pallor and petechiae over last 4 weeks. He also has
fevers during the same period. Vague history of URTI 2 months
prior with cough, runny nose and sore throat. Examination
showed a pale child, no hepatosplenomegaly. CBC: WBC 1.1 ×
109/L, Hb 5.2 g/dl, Platelets 7, ANC 120 (0.12 × 109/L). Bone
marrow Aspiration & Biopsy was done later and showed 75 %
hypocellular marrow. The most likely diagnosis:
A. Bone Marrow Suppression post viral infection
B. Diamond Blackfan Anemia
C. Fanconi Anemia
D. Severe Aplastic Anemia
E. Very severe Aplastic Anemia
71. Pediatric Board Review Hematology Oncology August 12, 2022
Aplastic Anemia
— Definition:
¡ Bone marrow failure disorder that is characterized by:
÷ Peripheral pancytopenia
÷ Bone marrow hypoplasia
¡ peripheral blood pancytopenia secondary to bone marrow hypocellularity
(<30%)
— Diagnosis Criteria for AA:
o Bone marrow hypocellular with no abnormal cells.
o At least 2 of the following:
o Hb <10 g/dL
o Platelet count <50 × 10^9/L
o Absolute neutrophil count <1.5 × 10^9/L
Killick SB, Bown N, Cavenagh J, et al; British Society for Standards in Haematology. Guidelines for the diagnosis and management of adult aplastic
anaemia. Br J Haematol. 2016;172:187-207
72. Pediatric Board Review Hematology Oncology August 12, 2022
Aplastic Anemia
Severity Classification (Gamitta’s Classification)
— Severe AA
• Bone marrow cellularity <25% or cellularity <50% with <30%
residual hematopoietic cells
• At least 2 of the following:
÷ Absolute reticulocyte count <1%
÷ Platelet count <20 × 10^9/L
÷ Absolute neutrophil count < 500 (0.5 × 10^9/L)
— Very severe AA
o Absolute neutrophil count < 200 (0.2 × 10^9/L)
o AND fulfils rest of the criteria for severe AA
— Non-severe AA
¡ Patients not fulfilling the criteria for severe or very severe AA
Camitta BM. Pathogenesis and treatment of aplastic anemia. Rinsho Ketsueki. 1984;25:459–469.
Samarasinghe, S., Veys, P., Vora, A. and Wynn, R. (2018), Paediatric amendment to adult BSH Guidelines for aplastic anaemia. Br J Haematol, 180: 201-205
73. Question 14
Pediatric Board Review Hematology Oncology August 12, 2022
— A 2 weeks old boy who just had a circumcision. He had a
massive bleeding 3 hours after the procedure. In ER, he was
investigated. Prothrombin (PT) was 12.3 (normal 11-14 seconds).
International Normalized Ratio (INR) was 1.1 (normal 1-1.2).
Activated Partial Thromboplastin Time (APTT) 119 seconds
(Normal 30-40 seconds). Factors FVIII and FIX levels requested
but are still pending. The most likely diagnosis is:
A. Factor VIII Deficiency
B. Factor II Deficiency
C. Factor V Deficiency
D. Factor XIII Deficiency
E. Von Willebrand Deficiency
74. Question 14
Pediatric Board Review Hematology Oncology August 12, 2022
— A 2 weeks old boy who just had a circumcision. He had a
massive bleeding 3 hours after the procedure. In ER, he was
investigated. Prothrombin (PT) was 12.3 (normal 11-14 seconds).
International Normalized Ratio (INR) was 1.1 (normal 1-1.2).
Activated Partial Thromboplastin Time (APTT) 119 seconds
(Normal 30-40 seconds). Factors FVIII and FIX levels requested
but are still pending. The most likely diagnosis is:
A. Factor VIII Deficiency
B. Factor II Deficiency
C. Factor V Deficiency
D. Factor XIII Deficiency
E. Von Willebrand Deficiency
75. Pediatric Board Review Hematology Oncology August 12, 2022
Hemophilia A
— FVIII Deficiency
— The most common inherited factor deficiency
— X-Linked recessive pattern
— Affects 1: 5000 boys
— Of all patients with Hemophilias, Hemophilia A represents about 85%
— Severe hemophilia is characterized by a factor level of less than < 1%
— Moderate hemophilia the clotting factor level is between 1 and 5 %
— Mild hemophilia FVIII or FIX levels are greater than 5 % but < 30%
Gitschier J, Wood WI, Goralka TM, et al.: Characterization of the human factor VIII gene. Nature. 312:326-330 1984
76. Pediatric Board Review Hematology Oncology August 12, 2022
Hemophilia A
— 70% are X-Linked Recessive inheritance
— 30 % are new mutations
— 10 % are qualitative dysfunction of FVIII
— 90% are quantitative
— Affects mainly males
— Females can be affected if they are homozygous
— Hemarthrosis (Joint bleeding), is the main site of bleeding
in 75% of patients
Goodeve A. Molecular genetic testing of hemophilia A. Semin Thromb Hemost. 2008;34:491-501. http://www.ncbi.nlm.nih.gov/pubmed/19085648
77. Pediatric Board Review Hematology Oncology August 12, 2022
Hemophilia B
— Factor IX deficiency
— The 2nd most common factor deficiency
— Major cause of morbidity and mortality in children
— Like all factor deficiencies, it is divided into:
¡ Mild > 5 %
¡ Moderate 1-5 %
¡ Severe <1%
— There is sometimes variation between level of factor IX
and the clinical picture of the disease
78. Pediatric Board Review Hematology Oncology August 12, 2022
Epidemiology of Hemophilia A & B
— Hemophilia A is more common than hemophilia B
— Hemophilia A is also more likely to be severe
— Hemophilia A –1 in 5000 live male births
¡ Approximately two-thirds have severe disease
(ie, factor VIII activity <1 percent of normal).
— Hemophilia B –1 in 20,000 live male births.
¡ Approximately half of them have severe disease
(ie, factor IX activity <1 percent of normal).
80. Pediatric Board Review Hematology Oncology August 12, 2022
Question 15
— A 3 weeks old girl who has recurrent bleeding from the umbilicus
as well as delayed detachment of the cord presented to your clinic
with yet another episode of bleeding from the umbilicus. You
assessed her and ordered some investigations. Hemoglobin was
8.4 g/dl, Platelet count was 290. PT was normal at 12 seconds
(normal 11-14). INR 1.1 (normal 1.0-1.1), PTT 33 seconds
(normal 30-40). The most likely diagnosis:
A. Factor XIII Deficiency
B. Factor VIII Deficiency
C. Von Willebrand Disease
D. Bernard Soulier Syndrome
E. Glanzmann’s Thrombosthenia
81. Pediatric Board Review Hematology Oncology August 12, 2022
Question 15
— A 3 weeks old girl who has recurrent bleeding from the umbilicus
as well as delayed detachment of the cord presented to your clinic
with yet another episode of bleeding from the umbilicus. You
assessed her and ordered some investigations. Hemoglobin was
8.4 g/dl, Platelet count was 290. PT was normal at 12 seconds
(normal 11-14). INR 1.1 (normal 1.0-1.1), PTT 33 seconds
(normal 30-40). The most likely diagnosis:
A. Factor XIII Deficiency
B. Factor VIII Deficiency
C. Von Willebrand Disease
D. Bernard Soulier Syndrome
E. Glanzmann’s Thrombosthenia
82. Pediatric Board Review Hematology Oncology August 12, 2022
Factor XIII Deficiency
— AR, Chromosome 6 , p25-p24
— “Delayed bleeding”
— The “Trauma today=bleeding tomorrow” factor J
— Fibrin stabilizing factor, Half life 5-7 days
— Other symptoms of factor XIII deficiency include:
• Delayed umbilical cord hemorrhage
• Delayed separation of the umbilical stump (beyond 4 weeks)
• Intracranial hemorrhage with little or no trauma
• Poor wound healing
• Recurrent spontaneous abortions in women
Muszbek L, Bereczky Z, Bagoly Z, et al.: Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev. 91 (3):931-972
83. Pediatric Board Review Hematology Oncology August 12, 2022
Question 16
— You are asked to assess a newborn girl with purpura fulminans.
Upon taking the medical history, you learn that this child had a
male sibling who died in the neonatal period after presenting with
purpura fulminans as well. On exam: she is vitally stable. You
noticed large areas of dark bluish discoloration of the skin with
bruising spots especially over upper and lower limbs. She has 3
other siblings who are healthy. Parents are consanguineous. What
is the most likely cause of this newborn purpura fulminans:
A. Sepsis
B. Factor XII Deficiency
C. Protein C Deficiency
D. Von Willebrand Disease
E. Meningitis
85. Pediatric Board Review Hematology Oncology August 12, 2022
Question 16
— You are asked to assess a newborn girl with purpura fulminans.
Upon taking the medical history, you learn that this child had a
male sibling who died in the neonatal period after presenting with
purpura fulminans as well. On exam: she is vitally stable. You
noticed large areas of dark bluish discoloration of the skin with
bruising spots especially over upper and lower limbs. She has 3
other siblings who are healthy. Parents are consanguineous. What
is the most likely cause of this newborn purpura fulminans:
A. Sepsis
B. Factor XII Deficiency
C. Protein C Deficiency
D. Von Willebrand Disease
E. Meningitis
86. Pediatric Board Review Hematology Oncology August 12, 2022
Etiology of “Neonatal Purpura Fulminans”
“A Common Pediatric Question”
— Congenital/inherited states
¡ 1. Homozygous protein C deficiency
¡ 2. Homozygous protein S deficiency
— Acquired causes
¡ Increased consumption:
1. Infection, e.g. group B streptococcus infection
2. Disseminated intravascular coagulation
3. Acute venous thrombosis
4. Antiphospholipid antibodies
5. Cardiac bypass
¡ Decreased synthesis:
6. Severe hepatic dysfunction
7. Galactosemia
8. Severe congenital heart disease
87. Physiology of Protein C
Pediatric Board Review Hematology Oncology August 12, 2022
What is the role of Protein C ?
88. Pediatric Board Review Hematology Oncology August 12, 2022
Protein C Deficiency
— AD, Protein C is 62-kD vitamin K-dependent glycoprotein
— The PROC gene is found on chromosome 2
— Synthesized in the liver as a single-chain zymogen
— Clipped into a serine-protease-like enzyme on phospholipid cell
surfaces by thrombin (aFII)
— Homozygous disease occurs in 1 out of every 200, 000 births
— Prognosis for Homozygous cases is usually poor.
— Can cause miscarriage or intrauterine thrombosis, or present soon
after birth.
— Symptoms are directly related to the degree of Protein C
deficiency
89. Pediatric Board Review Hematology Oncology August 12, 2022
Question 17
— A 7 year old girl who presented to ER with fever of 39 °C.
Physical exam showed height and weight below 5th
percentile. She also has small triangular face and a few café
au lait spots. CBC showed Hb of 6.2 g/dl, platelets count of
7, and Neutrophil count of 0.15 (ANC 150). What is the
most likely diagnosis?
A.Aplastic Anemia
B.Diamond Blackfan Syndrome
C.Schwachman-Diamond Synrome
D.Fanconi Anemia
E. Dyskeratosis Congenita
91. Pediatric Board Review Hematology Oncology August 12, 2022
Question 17
— A 7 year old girl who presented to ER with fever of 39 °C.
Physical exam showed height and weight below 5th
percentile. She also has small triangular face and a few café
au lait spots. CBC showed Hb of 6.2 g/dl, platelets count of
7, and Neutrophil count of 0.15 (ANC 150). What is the
most likely diagnosis?
A.Aplastic Anemia
B.Diamond Blackfan Syndrome
C.Schwachman-Diamond Synrome
D.Fanconi Anemia
E. Dyskeratosis Congenita
92. Pediatric Board Review Hematology Oncology August 12, 2022
Fanconi Anemia
— It is the most common inherited bone marrow failure syndrome
— AR, At least 23 genes are detected up to now
— 80-90% of cases are due to 3 genes: FANCA, FANCC, and FANCG
— 1:200 carrier status worldwide
— 1:70 in Spanish Gypsies and 1:77 in Ashkenazi Jews
— FA patients have a striking hypersensitivity to DNA interstrand cross-links
— Increase risk of malignancy (700 folds):
• MDS
• AML
• SCC
Justin Triemstra, MD; et al, A Review of Fanconi Anemia for the Practicing Pediatrician, Pediatr Ann. 2015;44(10):444-
445,448,450,452.
93. Pediatric Board Review Hematology Oncology August 12, 2022
Fanconi Anemia
— Skin (Café –Au-Lait spots)
— MSK:
÷ Thumbs (35%): absent or hypoplastic; supernumerary, bifid, duplicated
÷ Radii (7%): absent or hypoplastic
÷ Hands (5%): clinodactyly; hypoplastic thenar eminence
— Endocrine: Short stature, Hypothyroidism
— Eyes: Small eyes, strabismus, epicanthal folds, short or almond-shaped
palpebral fissures, hypertelorism
— Kidneys 20%: Ectopic or pelvic; abnormal, horseshoe, hypoplastic, or
dysplastic
Glanz A, Fraser FC: Spectrum of anomalies in Fanconi anaemia.J Med Genet. 19:412-416 1982
94. Pediatric Board Review Hematology Oncology August 12, 2022
Question 18
— You are evaluating a 12 year girl who was admitted to the
hospital with anemia due to significant vaginal bleeding.
Her onset of menarche was 3 weeks ago. Blood work and
CBC showed WBC 6.8 × 109/L , Hb 9.2 g/dl, Platelets 68,
PT 12.3, PTT 53.2, INR 1.1. What would be the most likely
diagnosis ?
A.Von Willebrand Disease type 1
B.Von Willebrand Disease type 2A
C.Von Willebrand Disease type 3
D.Von Willebrand Disease type 2B
E. Pseudo (platelet-type)-von Willebrand disease
95. Pediatric Board Review Hematology Oncology August 12, 2022
Question 18
— You are evaluating a 12 year girl who was admitted to the hospital
with anemia due to significant vaginal bleeding. Her onset of
menarche was 3 weeks ago. Blood work and CBC showed WBC
6.8 × 109/L , Hb 9.2 g/dl, Platelets 68, PT 12.3, PTT 33.2, INR
1.1. VWF Ag 77% (NR 60-120 %), Ristocetin Cofactor (VWF
Activity) 28%. Which of the following is the most likely
diagnosis ?
A. Von Willebrand Disease type 1
B. Von Willebrand Disease type 2A
C. Von Willebrand Disease type 3
D. Von Willebrand Disease type 2B
E. Pseudo (platelet-type)-von Willebrand disease
96. Pediatric Board Review Hematology Oncology August 12, 2022
Classification of VWD
A- Quantitative deficiency of VWF
Type 1: Partial quantitative deficiency of vWF
Type 1c: Increased clearance, ê levels of vWF
Type 3: Virtually complete deficiency of vWF
B- Qualitative deficiency of VWF
Type 2A: Qualitative variants with decreased platelet dependent function
associated with the absence of high and intermediate molecular
weight vWF multimers
Type 2B: Qualitative variants with increased affinity for platelet GPIb
Type 2M: Qualitative variants with decreased platelet dependent function
not caused by the absence of high-molecular weight vWF multimers
Type 2N: Qualitative variants with markedly decreased affinity for factor VIII
97. Pediatric Board Review Hematology Oncology August 12, 2022
Question 19
— A 3 year old boy was brought to the emergency room with pallor, dark red
urine and jaundice. He had a recent history of fevers and lower abdominal
pain, went to polyclinic, urinalysis showed +ve Nitrite and Urine Culture
showed E.coli and he was given Co-Trimoxazole antibiotic (Bactrim). 5 days
after, he became pale, jaundiced and urine became dark red.
— In ER, CBC showed WBC of 19.6 × 109/L , hemoglobin level of 5.2 g/dl,
platelets of 344. T-Bilirubin was 96 umol/L. LDH was 1156 U/L.
— Blood film showed Heinz bodies. Which of the following medications would
be safe to use in a child with G6PD:
A. Co-Trimoxazole
B. Nitrofurantoin
C. Cefixime
D. Vitamin C
E. Hydroxychloroquine
99. Pediatric Board Review Hematology Oncology August 12, 2022
Question 19
— A 3 year old boy was brought to the emergency room with pallor, dark red
urine and jaundice. He had a recent history of fevers and lower abdominal
pain, went to polyclinic, urinalysis showed +ve Nitrite and Urine Culture
showed E.coli and he was given Co-Trimoxazole antibiotic (Bactrim). 5 days
after, he became pale, jaundiced and urine became dark red.
— In ER, CBC showed WBC of 19.6 × 109/L , hemoglobin level of 5.2 g/dl,
platelets of 344. T-Bilirubin was 96 umol/L. LDH was 1156 U/L.
— Blood film showed Heinz bodies. Which of the following medications would
be safe to use in a child with G6PD:
A. Co-Trimoxazole
B. Nitrofurantoin
C. Cefixime
D. Vitamin C
E. Hydroxychloroquine
101. Pediatric Board Review Hematology Oncology August 12, 2022
Question 20
— A 6 year old girl who was diagnosed with Iron Deficiency
Anemia. Her initial Hemoglobin was 7.1, MCV 69 fl, MCH 19
pg. After 2 months course of Iron therapy, Her Hemoglobin is
still 7.2. Which one of these would explain the unresponsiveness
to treatment?
A. Non-Compliance
B. Malabsorption (e.g Celiac Disease)
C. Using Proton Pump Inhibitors PPI /Antacids
D. Iron Refractory Iron Deficiency Anemia (IRIDA)
E. All of the above
102. Pediatric Board Review Hematology Oncology August 12, 2022
Question 20
— A 6 year old girl who was diagnosed with Iron Deficiency
Anemia. Her initial Hemoglobin was 7.1, MCV 69 fl, MCH 19
pg. After 2 months course of Iron therapy, Her Hemoglobin is
still 7.2. Which one of these would explain the unresponsiveness
to treatment?
A. Non-Compliance
B. Malabsorption (e.g Celiac Disease)
C. Using Proton Pump Inhibitors PPI /Antacids
D. Iron Refractory Iron Deficiency Anemia (IRIDA)
E. All of the above
103. Pediatric Board Review Hematology Oncology August 12, 2022
Iron Deficiency Anemia
— The single most important nutritional deficiency in the world
— Children are the first & second age category for suffering from IDA
— Affects about 30% of Saudi children
— Can be a diagnostic dilemma
— Classic IDA Diagnosis: ↓ Hb, ↓ MCV, ↓ MCH, ↑ RDW, ↓ Iron, ↓ Ferritin, ↑ TIBC
— Best diagnostic marker is low ferritin
— sTfR is the best when patient is sick children who have high ferritin
— Retic-Hb is a cheap test & has a sensitivity of almost 100% for IDA
— If persistent, TMPRSS6 gene mutation should be suspected (IRIDA)
— Remember: management: non-pharmacological + pharmacologic
Luigia DeFalco, Mayka Sanchez, Laura Silvestri, Caroline Kannengiesser, MartinaMuckenthaler, Achille Iolascon, Laurent Gouya, Clara Camaschella, Carole Beaumont, Iron Refractory Iron
Deficiency Anemia. Haematologica June 2013 98: 845-853
104. Gelaw Y, Woldu B, Melku M. The Role of Reticulocyte Hemoglobin Content for Diagnosis of Iron
Deficiency and Iron Deficiency Anemia, and Monitoring of Iron Therapy: a Literature Review. Clin Lab.
2019;65(12):10.7754/Clin.Lab.2019.190315. doi:10.7754/Clin.Lab.2019.190315
Pediatric Board Review Hematology Oncology August 12, 2022
105. Pediatric Board Review Hematology Oncology August 12, 2022
AAP & Iron Deficiency Anemia In Children
1. AAP recommends screening for all children between 9-12 mo with Hgb or Hct.
2. Adjunct screening (ferritin or zinc protoporphyrin to heme ratio, ZPPH) for ID only
3. Rescreening is recommended 6-12 months later in high-risk populations
4. Additional screening for IDA should be considered for:
a. Adolescent females within a year of onset of menses
b. Children with special health considerations including:
i. Children with Restricted Diet
ii. Children with GI dysfunction
iii. Children with BMI >95th percentile
iv. Children with Restless Leg Syndrome
5. AAP recommends giving breastfed infants 1 mg/kg/day of a liquid Iron supplement until iron
containing solid food is introduced.
106. Pediatric Board Review Hematology Oncology August 12, 2022
Question 21
— A 3 day old infant is brought to the ER due to a seizure. A
CT scan demonstrates massive intracranial hemorrhage. On
your examination, the child has numerous bruises on the
abdomen and trunk. No sepsis risk factors are present. PT,
PTT and INR are all prolonged, Fibrinogen normal. Platelets
count 322. Which of the below scenarios is most likely:
A.The baby was born to an infant of a diabetic mother.
B.The baby was born at home
C.The baby is exclusively breastfed.
D.The baby has trauma
E. The baby was born to a hypothyroidism mother
107. Pediatric Board Review Hematology Oncology August 12, 2022
Question 21
— A 3 day old infant is brought to the ER due to a seizure. A
CT scan demonstrates massive intracranial hemorrhage. On
your examination, the child has numerous bruises on the
abdomen and trunk. No sepsis risk factors are present. PT,
PTT and INR are all prolonged, Fibrinogen normal. Platelets
count 322. Which of the below scenarios is most likely:
A.The baby was born to an infant of a diabetic mother.
B.The baby was born at home
C.The baby is exclusively breastfed.
D.The baby has trauma
E. The baby was born to a hypothyroidism mother
108. Pediatric Board Review Hematology Oncology August 12, 2022
Hemorrhagic Disease of The Newborn
— Early Onset HrDN:
¡ Occurs within 24 hrs
¡ Usually due to maternal meds; antiepileptics, certain antibiotics, anticoagulants (W)
— Classical HrDN: (2-7 days)
¡ Typically, in those who did not receive Vitamin K Prophylaxis (Home Delivery)
— Late HrDN (1-6 mo)
o Commonly in; Malabsorption , Hepatitis , biliary atresia , Cystic fibrosis, Alpha1-
antitrypin deficiency, Short bowel syndrome, Intestinal bacterial overgrowth, Chronic
exposure to broad spectrum antimicrobials
o Low levels of vitamin K and subsequent low vitamin K–dependent clotting factors.
Bör, Ö., Akgün, N., Yakut, A., Sarhuş, F. and Köse, S. (2000), Late hemorrhagic disease of the newborn. Pediatrics International, 42: 64–66.
doi:10.1046/j.1442-200x.2000.01173
109. Pediatric Board Review Hematology Oncology August 12, 2022
Question 22
— A full term baby boy at 1 day age was noticed in nursery to be very
jaundiced. T-Bilirubin was measured, and it was 398 μmol/L . CBC
showed Hb 7.9 g/dl. Direct Antiglobulin Test DAT (Coomb’s Test) was
strong positive (4 +). He was transferred to NICU. Started on
Phototherapy, IVIG and double volume exchange transfusion.
— Mother is G2P1, O- but received Rhogam (Anti-D on 28 week & at 34
week of GA). Baby was O+. Which of the below scenarios is most
likely:
A. Hemolytic Disease of Newborn Due to Rh D
B. Hemolytic Disease of Newborn Due to ABO Incompatibility
C. Hemolytic Disease of Newborn Due to probably other Rh antigens
D. Hemolytic Disease of Newborn due Duffy antigen
E. Baby probably has autoimmune hemolytic anemia
110. Pediatric Board Review Hematology Oncology August 12, 2022
Question 22
— A full term baby boy at 1 day age was noticed in nursery to be very jaundiced. T-
Bilirubin was measured, and it was 398 μmol/L . CBC showed Hb 7.9 g/dl.
Direct Antiglobulin Test DAT (Coomb’s Test) was strong positive (4 +). He was
transferred to NICU. Started on Phototherapy, IVIG and double volume
exchange transfusion.
— Mother is G2P1, O- but received Rhogam (Anti-D on 28 week & at 34 week of
GA). Baby was O+. Which of the below scenarios is most likely:
A. Hemolytic Disease of Newborn Due to Rh D
B. Hemolytic Disease of Newborn Due to ABO Incompatibility
C. Hemolytic Disease of Newborn Due to probably other Rh antigens
D. Hemolytic Disease of Newborn due Duffy antigen
E. Baby probably has autoimmune hemolytic anemia
111. Hemolytic Disease of Newborn (HDN)
August 12, 2022
Pediatric Board Review Hematology Oncology
— HDN is a potentially fatal disease
— Caused by immune destruction of fetal RBCs via transplacentally acquired maternal antibodies
— Incidence – 6-7/1,000 live births in U.S. (CDC 2020)
• Dramatic decrease in cases since introduction of anti-D immunoglobulin
— Most involved Antigens:
• Anti-Rh (D, C, c, E, and e)
• ABO
• Anti-Kell (K and k)
• Anti-Duffy (Fya)
• Anti-Kidd (Jka and Jkb)
— Treatment:
• Phototherapy
• Exchange transfusion in severe cases
• IVIG / Corticosteroids
• PRBCs
Isabelle M. C. Ree, Vivianne E. H. J. Smits-Wintjens, Johanna G. van der Bom, Jeanine M. M. van Klink, Dick Oepkes & Enrico Lopriore (2017) Neonatal management and outcome in alloimmune hemolytic
disease, Expert Review of Hematology, 10:7, 607-616
112. Prevalence of Rh Antigens
August 12, 2022
Pediatric Board Review Hematology Oncology
— The Rh genes are 97% identical
— Located next to each other on chromosome 1.
— Highly Immunogenic
— D: 85% Caucasians, 92% Blacks, 99% Asians
C: 68% Caucasians, 27% Blacks, 93% Asians
E: 29% Caucasians, 22% Blacks, 39% Asians
c: 80% Caucasians, 96% Blacks, 47% Asians
e: 98% Caucasians, 98% Blacks, 96% Asians
113. Pediatric Board Review Hematology Oncology August 12, 2022
Question 23
— A 2 days old newborn started to have a seizure that lasted about 20 minutes and
received Phenobarbital infusion. CBC showed Hb of 16.2 g/dl, Platelets of 4, WBC
11.7 × 109/L . Maternal Platelets was 346. No mottling, no respiratory symptoms.
Immediate CT scan showed moderate intracranial hemorrhage. IVIG was given and
showed a good response and platelets increased to 72. What is the most likely
diagnosis:
A. Disseminated Intravascular Coagulopathy (DIC)
B. Congenital Thrombocytopenia
C. Maternal ITP
D. Neonatal Alloimmune Thrombocytopenia (NAIT)
E. Drug Induced Thrombocytopenia
114. Pediatric Board Review Hematology Oncology August 12, 2022
Question 23
— A 2 days old newborn started to have a seizure that lasted about 20 minutes and
received Phenobarbital infusion. CBC showed Hb of 16.2 g/dl, Platelets of 4, WBC
11.7 × 109/L . Maternal Platelets was 346. No mottling, no respiratory symptoms.
Immediate CT scan showed moderate intracranial hemorrhage. IVIG was given and
showed a good response and platelets increased to 72. What is the most likely
diagnosis:
A. Disseminated Intravascular Coagulopathy (DIC)
B. Congenital Thrombocytopenia
C. Maternal ITP
D. Neonatal Alloimmune Thrombocytopenia (NAIT)
E. Drug Induced Thrombocytopenia
115. Neonatal Alloimmune Thrombocytopenia
August 12, 2022
Pediatric Board Review Hematology Oncology
— NAIT : platelet destruction due to maternal Abs against fetal platelets antigens
— Parents are incompatible for human platelet antigens HPA1a or HPA5b.
— NAIT should be strongly suspected in ??
— In contrast to HDN, the first-born child is often affected with NAIT
— Accurate diagnosis is essential to provide appropriate care to the affected neonate
Peterson, J. A., McFarland, J. G., Curtis, B. R. and Aster, R. H. (2013), Neonatal alloimmune thrombocytopenia: pathogenesis, diagnosis and
management. Br J Haematol, 161: 3–14. doi:10.1111/bjh.12235
116. Pediatric Board Review Hematology Oncology August 12, 2022
Question 24
— A 5 year old girl presented with recent history of upper
respiratory tract infection about 3 weeks ago. Now she presented
with severe epistaxis and bruises all over her body. CBC showed
WBC 12.3 × 109/L, Hemoglobin of 11.4 g/dl, Platelets of 4. She
is Group O negative. The first line therapy for management of
this child is:
A. IVIG
B. Rituximab
C. Anti-D
D. Eltrombopag
E. Romiplostim
117. Pediatric Board Review Hematology Oncology August 12, 2022
Question 24
— A 5 year old girl presented with recent history of upper
respiratory tract infection about 3 weeks ago. Now she presented
with severe epistaxis and bruises all over her body. CBC showed
WBC 12.3 × 109/L, Hemoglobin of 11.4 g/dl, Platelets of 4. She
is Group O negative. The first line therapy for management of
this child is:
A. IVIG
B. Rituximab
C. Anti-D
D. Eltrombopag
E. Romiplostim
118. Pediatric Board Review Hematology Oncology August 12, 2022
Idiopathic Thrombocytopenia Pupura (ITP)
— ITP is the most common Pediatric Platelet Disorder
— Immune Mediated Thrombocytopenia
— Often follows a viral illness or immunization
— Some Children with ITP require no treatment
— 80-85% resolve within 6 months
— 15-20 % progress to chronic ITP
— “Serious Bleeding” in Acute ITP is RARE but it can happen
119. Pediatric Board Review Hematology Oncology August 12, 2022
Clinical Manifestations
— Petechiae, Ecchymosis, and purpura
— Nosebleed
— GI / GU Bleeding
— ICH
120. Pediatric Board Review Hematology Oncology August 12, 2022
Diagnosis of ITP
—Clinical Diagnosis: Hx and P/E
—CBC
—Blood film
—Bone Marrow: Indications ??
121. Pediatric Board Review Hematology Oncology August 12, 2022
Risk Factors for Serious Bleeding / ICH in Children with ITP
1. Extreme Thrombocytopenia: Plts < 10 in 75% of children with ICH.
2. Timing: 50% children developed ICH within 7 days of diagnosis of ITP
3. Head Trauma
4. Hematuria
5. NSAID Use (When ?)
6. Presence of Cerebral AVM
Bethan Psaila et al, 2009 114: 4777-4783, Prepublished online September 18, 2009; Blood Journal
122. Pediatric Board Review Hematology Oncology August 12, 2022
Predictors of Chronicity in Pediatric ITP
1. Age
2. Female gender
3. Platelet count at presentation
4. Absence of mucosal bleeding with low platelet count at diagnosis
5. No history of preceding upper respiratory tract infection
6. Recent MMR vaccine
7. ANA positivity
Heitink-Pollé KM, Nijsten J, Boonacker CW, de Haas M, Bruin MC. Clinical and laboratory predictors of chronic immune thrombocytopenia in
children:
a systematic review and meta-analysis. Blood. 2014 Nov 20. 124(22):3295-307
123. Pediatric Board Review Hematology Oncology August 12, 2022
Management of ITP
— No treatment
— IVIG
— Corticosteroids
— Anti-D
— Other treatments
— Role of TPO-RA in Children
124. Thrombopoietin Agonists
— Eltrombopag: (Promacta Novartis) Approved by FDA 10 July 2015
¡ Children with chronic ITP
¡ Who are older than 6 year
¡ Have ITP for 12 months
¡ Who have insufficient response to corticosteroids, IVIG, or splenectomy.
— Romiplostim: (Nplate Amgen) Approved by FDA 14 December 2018 for
¡ Children with ITP
¡ Who are older than 1 year
¡ Have ITP for at least 6 mo
¡ Who have insufficient response to corticosteroids, IVIG, or splenectomy.
125. Pediatric Board Review Hematology Oncology August 12, 2022
Question 25
— Which of the following is not associated with Leukemoid
Reaction in children (WBC count >50,000/μL)
A. Shigellosis
B. Septicemia
C. Salmonellosis
D. Chronic Granulomatous Disease (CGD)
E. Leukocyte Adhesion Deficiency
126. Pediatric Board Review Hematology Oncology August 12, 2022
Question 25
— Which of the following is not associated with Leukemoid
Reaction in children (WBC count >50,000/μL)
A. Shigellosis
B. Septicemia
C. Salmonellosis
D. Chronic Granulomatous Disease (CGD)
E. Leukocyte Adhesion Deficiency
127. Pediatric Board Review Hematology Oncology August 12, 2022
Leukemoid Reaction
— Definition: ↑ in the WBC count, which can mimic leukemia.
— 3 broad differential:
¡ Infectious Process
¡ Leukemic Process
¡ Leukocyte adhesion syndrome
— What will make it likely infectious ?
— Leukocyte alkaline phosphatase (LAP)
Vissaria Sakka, Sotirios Tsiodras, Evangelos J. Giamarellos-Bourboulis, Helen Giamarellou, An update on the etiology and
diagnostic evaluation of a leukemoid reaction, European Journal of Internal Medicine 17 (2006) 394–398
128. Pediatric Board Review Hematology Oncology August 12, 2022
Question 26
— A 9 year old boy presented with severe pallor. He was also
found on physical examination to have splenomegaly.
— CBC showed WBC of 9.7 × 109/L , Hemoglobin of 4.2,
Platelets count of 344. Reticulocyte count was 9.4%. Direct
Coomb’s Test was positive 3+ IgG. The first line in
management of this condition is:
A.Corticosteroids
B.IVIG
C.Splenectomy
D.Packed Red Blood Transfusion (PRBCs)
E. Eryhtropoietin
129. Pediatric Board Review Hematology Oncology August 12, 2022
Question 26
— A 9 year old boy presented with severe pallor. He was also
found on physical examination to have splenomegaly.
— CBC showed WBC of 9.7 × 109/L , Hemoglobin of 4.2,
Platelets count of 344. Reticulocyte count was 9.4%. Direct
Coomb’s Test was positive 3+ IgG. The first line in
management of this condition is:
A.Corticosteroids
B.IVIG
C.Splenectomy
D.Packed Red Blood Transfusion (PRBCs)
E. Eryhtropoietin
131. Pediatric Board Review Hematology Oncology August 12, 2022
Question 27
— A strictly vegan 12 years old girl presented to your clinic
with pallor, irritability, loss to taste, CBC showed WBC
2.2 x 109/L, Hb 6.2, MCV 110 fL, MCH 17, MCHC 29
%. Platelets 92. Blood film showed hypersegmented
neutrophils (> 5 segments). LDH 944 U/L. What is the
most likely diagnosis:
A. Iron Deficiency Anemia
B. Vitamin B 12 Deficiency
C. Vitamin D Deficiency
D. Vitamin B1 Deficiency
E. Vitamin B6 Deficiency
132. Pediatric Board Review Hematology Oncology August 12, 2022
Question 27
— A strictly vegan 12 years old girl presented to your clinic
with pallor, irritability, loss to taste, CBC showed WBC
2.2 x 109/L, Hb 6.2, MCV 110 fL, MCH 17, MCHC 29
%. Platelets 92. Blood film showed hypersegmented
neutrophils (> 5 segments). LDH 944 U/L. What is the
most likely diagnosis:
A. Iron Deficiency Anemia
B. Vitamin B 12 Deficiency
C. Vitamin D Deficiency
D. Vitamin B1 Deficiency
E. Vitamin B6 Deficiency
133. Vitamin B12
August 12, 2022
Pediatric Board Review Hematology Oncology
1.Vitamin B12 is a family of related compounds containing a
cobalt atom (cobalamins).
2.The two dietary forms of vitamin B12 are available and they
are known as methylcobalamin (meth yl-B12) and 5-
deoxyadenosylcobalamin (coenzyme-B12).
3.Synthetic forms of vitamin B12 are known as hydroxy-
cobalamin and cyanocobalamin (not occur naturally in
foods).
4.Structure of B12 is very complicated and based on a corrin
ring, similar to porphyrin ring found in heme, chlorophyll,
and cytochrome.
134. Absorption of vitamin B12
August 12, 2022
Pediatric Board Review Hematology Oncology
vVitamin B12 in food is bound to protein.
v HCl of the stomach releases the free vitamin B12.
vOnce released, vitamin B12 combines with glycoprotein
intrinsic factor (IF) secreted by the parietal cells of
stomach to form a complex which can be absorbed from
ileum.
135. Functions of Vitamin B12
August 12, 2022
Pediatric Board Review Hematology Oncology
1. Essential with folic acid in RBCs maturation (it protects
against Pernicious anemia).
2. Folate metabolism. Vitamin B12 is vital in activation of
folate to the active THF. In vitamin B12 deficiency, tissue
stores of folate are “trapped” as inactive methylated forms,
and a functional folate deficiency results.
3. Protein metabolism: Act as coenzyme with THF in the
synthesis of methionine from homocysteine.
136. Functions of Vitamin B12
August 12, 2022
Pediatric Board Review Hematology Oncology
— It is vital in fat metabolism.
— Helps maintain the antioxidant status by maintaining
glutathione in the reduced form.
— Nervous system (It is vital in synthesis of myelin sheath of
neurons).
— Cell replication. It is essential with THF in synthesis of
nucleic acids
137. Diagnosis of Vitamin B 12
Pediatric Board Review Hematology Oncology August 12, 2022
1. Measurement of serum Vitamin B12
2. MMA level
3. Schilling Test
138. Pediatric Board Review Hematology Oncology August 12, 2022
Question 28
— A 2 yr old boy presented to your clinic with pallor. Mother
reported drinking a lot of fresh “Goat Milk”. She said she
has been giving him this milk since he was 9 months of
age. You order CBC and WBC 6.7 x 109/L , Hb 8.1 g/dl,
MCV 107 fL, MCH 19 pg, MCHC 26 %. What test would
confirm the diagnosis for this condition:
A. Serum Folate
B. Iron Level
C. LDH
D. Ferritin
E. RBC Folate
139. Pediatric Board Review Hematology Oncology August 12, 2022
Question 28
— A 2 yr old boy presented to your clinic with pallor. Mother
reported drinking a lot of fresh “Goat Milk”. She said she
has been giving him this milk since he was 9 months of
age. You order CBC and WBC 6.7 x 109/L , Hb 8.1 g/dl,
MCV 107 fL, MCH 19 pg, MCHC 26 %. What test would
confirm the diagnosis for this condition:
A. Serum Folate
B. Iron Level
C. LDH
D. Ferritin
E. RBC Folate
140. Pediatric Board Review Hematology Oncology August 12, 2022
Folate Deficiency
— Abdorbtion:
¡ Folate is absorbed in the jejunum by active and passive transport mechanisms across the
intestinal wall.
— Etiology:
¡ Malabsorption: Celiac disease, short bowel syndrome, Gastric Bypass
¡ Amyloidosis
¡ Hypothyroidism
¡ Goat Milk ?
¡ Drugs such as
÷ methotrexate
÷ phenytoin
÷ Sulfasalazine
÷ Trimethoprim
— Diagnosis:
÷ Serum Folate: <2 ng/mL are considered deficient, while levels > 4 ng/ml are considered as
normal
÷ RBC Folate: > 160 ng/mL is considered normal
— Treatment: oral folic acid (1 to 5 mg daily) typically for 1-3 months
142. Pediatric Board Review Hematology Oncology August 12, 2022
Question 29
— A 2 yr old boy presented with incidentally found abdominal
mass. While he was being bathed, the mother felt a palpable
abdominal mass on the left side. CT abdomen showed a
homogenous abdominal mass, does not cross the midline, with
no calcification that has an area of necrosis and originate from
the left kidney. Patient has also the lesion shown on the photo.
What is the most likely diagnosis:
A. Wilms Tumor
B. Neuroblastoma
C. Rhabdomyosarcoma
D. Hepatolastoma
E. Adrenal Cell Carcinoma
145. Pediatric Board Review Hematology Oncology August 12, 2022
Question 29
— A 2 yr old boy presented with incidentally found abdominal
mass. While he was being bathed, the mother felt a palpable
abdominal mass on the left side. CT abdomen showed a
homogenous abdominal mass, does not cross the midline, with
no calcification that has an area of necrosis and originate from
the left kidney. Patient has also the lesion shown on the photo.
What is the most likely diagnosis:
A. Wilms Tumor
B. Neuroblastoma
C. Rhabdomyosarcoma
D. Hepatolastoma
E. Adrenal Cell Carcinoma
146. Pediatric Board Review Hematology Oncology August 12, 2022
Wilms Tumor
— Embryonal tumor develops from remnants of Immature
Kidney Tissue
— Survival has improved from 30% in the 1930’s to over 85%
currently
— Current management now emphasizes reducing morbidity of
treatment for low-risk patients
— More intensive treatment for selected high-risk patients for
whom survival remains poor.
147. n Wilms tumor was named after
German surgeon Max Wilms,
who published the first
comprehensive review of the
disease in 1899.
n The National Wilms Tumor Study
Group (NWTSG) established in
1969.
Pediatric Board Review Hematology Oncology August 12, 2022
Wilms Tumor
148. Pediatric Board Review Hematology Oncology August 12, 2022
Question 30
— A 6 year old girl presented with 6 weeks history of progressive pallor, fatigue and loss
of energy. She lost about 3 kg over the same period of time. CBC showed WBC 244.8
× 109/L , Hemoglobin 7.3 g/dl, Platelets of 34. She has petechial rash and significant
hepatosplenomegaly. Also was found to have Uric acid of 845 umol/L, K 7.6 mEq/L,
Phosphate of 2.9 mmol/L. Mg .051 mmol/L. Renal profile showed Creatinine of 256
umol//L, BUN of 15.7 mmol/L. Which of her conditions need immediate management?
A. Hyperuricemia, hyperkalemia, hyperphosphatemia
B. Renal failure (Dialysis)
C. Hyperleukocytosis (IV Fluids & Leukophresis)
D. Hypomagnesemia
E. A, B, and C
149. Pediatric Board Review Hematology Oncology August 12, 2022
Question 30
— A 6 year old girl presented with 6 weeks history of progressive pallor, fatigue and loss
of energy. She lost about 3 kg over the same period of time. CBC showed WBC 244.8
× 109/L , Hemoglobin 7.3 g/dl, Platelets of 34. She has petechial rash and significant
hepatosplenomegaly. Also was found to have Uric acid of 845 umol/L, K 7.6 mEq/L,
Phosphate of 2.9 mmol/L. Mg .051 mmol/L. Renal profile showed Creatinine of 256
umol//L, BUN of 15.7 mmol/L. Which of her conditions need immediate management?
A. Hyperuricemia, hyperkalemia, hyperphosphatemia
B. Renal failure (Dialysis)
C. Hyperleukocytosis (IV Fluids & Leukophresis)
D. Hypomagnesemia
E. A, B, and C
150. Laboratory Tumor Lysis Syndrome
Metabolite or electrolyte Criterion for diagnosis
Uric acid >475 umol/L or 25% increase from baseline
Potassium >6 mEq/L or 25% increase from baseline
Phosphorus > 6.5 mg/dL (children) or 25% increase from
baseline
Calcium
25% decrease from baseline
Cairo MS, Bishop M. Tumor lysis syndrome: New therapeutic strategies and classification. Br J Haematol 127: 3–11, 2004
Pediatric Board Review Hematology Oncology August 12, 2022
Cairo-Bishop Definition of Laboratory Tumor Lysis syndrome
151. Pediatric Board Review Hematology Oncology August 12, 2022
Cairo-Bishop Definition of Clinical Tumor Lysis Syndrome
— Clinical Tumor Lysis Syndrome: LTLS and one or more of:
1. Renal Insufficiency: ( Creatinine > 1.5 ULN)
2. Cardiac arrhythmia
3. Seizures
Cairo MS, Bishop M. Tumor lysis syndrome: New therapeutic strategies and classification. Br J Haematol 127: 3–11, 2004
152. Pediatric Board Review Hematology Oncology August 12, 2022
Question 31
— A 9 mo old boy presented with diarrhea and on
examination he has an abdominal mass that crosses the
midline. Vital signs are normal. History of decreased
appetite and 10% weight loss. You ordered work up and
CT abdomen showed heterogenous, lobulated soft tissue
mass with calcification. The most likely Diagnosis:
A. Wilms Tumor
B. Neuroblastoma
C. Rhabdomyosarcoma
D. Hepatolastoma
E. Adrenal Cell Carcinoma
153. 9 mo old boy with
abdominal mass
Pediatric Board Review Hematology Oncology August 12, 2022
154. Pediatric Board Review Hematology Oncology August 12, 2022
Question 31
— A 9 mo old boy presented with diarrhea and on
examination he has an abdominal mass that crosses the
midline. Vital signs are normal. History of decreased
appetite and 10% weight loss. You ordered work up and
CT abdomen showed heterogenous, lobulated soft tissue
mass with calcification. The most likely Diagnosis:
A. Wilms Tumor
B. Neuroblastoma
C. Rhabdomyosarcoma
D. Hepatolastoma
E. Adrenal Cell Carcinoma
155. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma
— Malignancy of infants and young children
— Arise from neural crest cells that give rise to
sympathetic ganglia
— One of the Small Round Blue Cell Tumors (SRBCT)
— Wide variety of outcomes
156. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma
— A spectrum of Neuroblastic Tumors:
¡ Ganglioneuromas
¡ Ganglioneuroblastomas
¡ Neuroblastoma
157. Pediatric Board Review Hematology Oncology August 12, 2022
Epidemiology of Neuroblastoma
Incidence
— 65 per million in infants <1 yr of age
— 30 per million in second yr of life
— 9 per million in children < 15 yr of age
— 90 % of all cases occur before 10 yrs of age
— Neuroblastoma is the most common cancer occurring in the first yr of life
Ries et al SEER1994
158. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma
— Neuroblastomas account for 97% of all neuroblastic tumors
— They are heterogeneous, varying in terms of:
¡ location
¡ histopathologic appearance
¡ biologic characteristics
— Broad spectrum of clinical behavior, which can range from:
¡ Spontaneous regression
¡ Maturation to a benign ganglioneuroma
¡ Aggressive disease with metastatic dissemination leading to
death
159. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma Clinical Course
Two distinct entities:
— Infant:
1. Possibility of spontaneous regression (apoptosis or differentiation
into ganglioneuroblastoma)
2. Chemosensitive
3. Chemocurable
— Older
¡ Aggressive tumor
¡ Chemoresistant
160. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma; Clinical Presentation
— Abdominal mass (retroperitoneal or hepatic)
— Abdominal pain or constipation
— Rib Cage Pain/ Back Pain (Paraspinal location / posterior
mediastinum)
— Proptosis
— Periorbital ecchymoses ("raccoon eyes", from periorbital
ecchymosis caused by orbital metastases)
— Horner syndrome (miosis, ptosis, anhidrosis)
— Localized back pain, weakness (from spinal cord compression)
— Scoliosis, bladder dysfunction
— Palpable nontender subcutaneous nodules
161. Pediatric Board Review Hematology Oncology
A. Subcutaneous Noduels B. Racoon Eyes
August 12, 2022
162. Pediatric Board Review Hematology Oncology August 12, 2022
Neuroblastoma
— Management:
¡ Surgery
¡ Chemotherapy
¡ Radiation Therapy
— Prognosis:
¡ Age:
÷ Age younger than 1 year: 90%.
÷ Age 1 to 4 years: 68%.
÷ Age 5 to 9 years: 52%.
÷ Age 10 to 14 years: 66%
¡ MYCN Amplication
163. Pediatric Board Review Hematology Oncology August 12, 2022
Question 32
— A 6 year old girl previously healthy presented to ER with history of pallor, bone pain
and lately fevers. On physical examination, she looks pale and tired. T 39.2, HR 125,
BP 110/65. RR 14 and O2 Sats 99% on R/A. She had multiple significant cervical
lymphadenopathy. Also Spleen was 6 cm BCM and liver 5 cm BCM. CBC showed
WBC of 66.4 × 109/L , Hemoglobin of 7.8, Platelets count of 76. LDH 1855 IU/L. ALT
77 IU/L, AST 92 IU/L. Urea 3.5 mg/ml and Creatinine 45 μmol/L. Chest X-Ray
showed a mediastinal mass. What is the most likely diagnosis ?
A. Pre-B Acute Lymphoblastic Leukemia (Pre-B ALL)
B. T-Cell Acute Lymphoblastic Leukemia (T-Cell ALL)
C. Acute Myeloid Leukemia (AML)
D. Chronic Lymphoblastic Leukemia (CML)
E. Juvenile Myelomonocytic Leukemia (JMML)
164. Pediatric Board Review Hematology Oncology August 12, 2022
Question 32
— A 6 year old girl previously healthy presented to ER with history of pallor, bone pain
and lately fevers. On physical examination, she looks pale and tired. T 39.2, HR 125,
BP 110/65. RR 14 and O2 Sats 99% on R/A. She had multiple significant cervical
lymphadenopathy. Also Spleen was 6 cm BCM and liver 5 cm BCM. CBC showed
WBC of 66.4 × 109/L , Hemoglobin of 7.8, Platelets count of 76. LDH 1855 IU/L. ALT
77 IU/L, AST 92 IU/L. Urea 3.5 mg/ml and Creatinine 45 μmol/L. Chest X-Ray
showed a mediastinal mass. What is the most likely diagnosis ?
A. Pre-B Acute Lymphoblastic Leukemia (Pre-B ALL)
B. T-Cell Acute Lymphoblastic Leukemia (T-Cell ALL)
C. Acute Myeloid Leukemia (AML)
D. Chronic Lymphoblastic Leukemia (CML)
E. Juvenile Myelomonocytic Leukemia (JMML)
165. — ALL is the most common cancer diagnosed in children
— About 25%-40% of “cancer diagnoses” among children <15 years.
— Higher in KSA .. Why ??
— ALL occurs at an annual rate of approximately 30 to 40 per million.
— 2,400 children and adolescents < 20 years diagnosed with ALL /yr
— Gradual increase in the incidence of ALL in the past 25 years
— harp peak in ALL incidence is observed among children aged 2 to 3 y
Ries LA, Kosary CL, Hankey BF, et al., eds.: SEER Cancer Statistics Review, 1973-1996. Bethesda, Md: National Cancer Institute, Feb 2, 2012
Shah A, Coleman MP: Increasing incidence of childhood leukaemia: a controversy re-examined. Br J Cancer 97 (7): 1009-12, 2007
Pediatric Board Review Hematology Oncology August 12, 2022
Pediatric Acute Lymphoblastic Leukemia
166. — The incidence of ALL among children aged 2 to 3 years:
¡ >90 cases per 1 million per year
¡ Fourfold greater than that for infants
¡ Tenfold greater than that for adolescents who are 19 years old.
— ALL is substantially higher in white children than in black children
— Incidence is substantially higher in white children than in black children
Pediatric Board Review Hematology Oncology August 12, 2022
Pediatric Acute Lymphoblastic Leukemia
167. } Signs & Symptoms: reflect bone marrow infiltration and/or extramedullary
disease.
} When leukemic blasts replace the bone marrow, patients present with signs of ??
} Bone pain, arthritis, and limping may be presenting symptoms and in 5% of
patients are the only symptoms
} Fevers are common at presentation, but despite neutropenia, sepsis is rarely
seen.
} Other common clinical manifestations include fatigue, pallor, petechiae, and
bleeding.
} Leukemic spread may manifest as lymphadenopathy and hepatosplenomegaly.
Pediatric Board Review Hematology Oncology August 12, 2022
Clinical Presentation
168. Clinical Presentation
Pediatric Board Review Hematology Oncology August 12, 2022
— Mature-B ALL : extramedullary masses in the abdomen or head and neck and
CNS involvement.
— In patients with T-lineage ALL, Respiratory distress ? renal failure. Why ??
— Symptoms of CNS involvement (headaches, vomiting, lethargy, and nuchal
rigidity: rare in B-Cell ALL
— More common in T-lineage and mature B cell ALL
— Testicular involvement at diagnosis is also rare; if present, it appears as
unilateral painless testicular enlargement. Genital exam in boys is a MUST
Pui CH, Robison LL, Look AT. Acute lymphoblastic leukaemia. Lancet. Mar 22 2008;371(9617):1030-43.
169. Pediatric Board Review Hematology Oncology August 12, 2022
Question 33
— You are a senior resident on the pediatric ward at KFAFH.
At 2 am, a nurse called you and told you Sarah, the 3 year
old girl with ALL in induction, has spiked a fever and she is
also neutropenic. What would predict the worst outcome:
A.Degree of Neutropenia
B.Duration of Neutropenia
C.Fever 39.6
D.Presence of cough
E.Presence of diarrhea
170. Pediatric Board Review Hematology Oncology August 12, 2022
Question 33
— You are a senior resident on the pediatric ward at KFAFH.
At 2 am, a nurse called you and told you Sarah, the 3 year
old girl with ALL in induction, has spiked a fever and she is
also neutropenic. What would predict the worst outcome:
A.Degree of Neutropenia
B.Duration of Neutropenia
C.Fever 39.6
D.Presence of cough
E.Presence of diarrhea
171. Pediatric Board Review Hematology Oncology August 12, 2022
Febrile Neutropenia
— Definition: ANC Calculation
— Why do we care ?
— Because infections in FN is rapidly fatal if not managed
properly
§ Mortality rate in the 1960’s was ~ 50%
§ With proper management < 5% today
Sung L, Phillips R, Lehrnbecher T: Time for paediatric febrile neutropenia guidelines: Children are not little adults. Eur J Cancer 47:811-813, 2011
172. Pediatric Board Review Hematology Oncology August 12, 2022
Assessment of FN Pediatric Oncology Patient
— Good history and physical exam
§ Be aware that with ¯ ANC may not have inflammation -
so redness, swelling and infiltrates may not be seen
§ Mouth, pharynx, lower esophagus, lung, skin, anus and
perineum are often sites of infection
— Blood work - CBC, Creat, BUN, liver profile, CRP
— Culture
§ blood cultures (include central line if present)
§ Urine / CSF / other cultures as indicated clinically
§ Chest X-Ray If Resp Symptoms present
Thomas Lehrnbecher, Paula Robinson, Sarah Alexander, and Lillian Sung et al, Guideline for the Management of Fever and Neutropenia in Children With Cancer and
Hematopoietic Stem-Cell Transplantation Recipients: 2017 Update, Journal of Clinical Oncology 2017 35:18, 2082-2094
174. Question 34
August 12, 2022
Pediatric Board Review Hematology Oncology
— Which of the following is not an anterior mediastinal tumor?
A. Thymoma
B. Lymphoma
C. Neurogenic Tumor
D. Teratoma
E. Thyroid Cancer
176. Question 34
August 12, 2022
Pediatric Board Review Hematology Oncology
— Which of the following is not an anterior mediastinal tumor?
A. Thymoma
B. Lymphoma
C. Neurogenic Tumor
D. Teratoma
E. Thyroid Cancer
177. Most common types of
mediastinal tumors in children
Pediatric Board Review Hematology
Oncology
August 12, 2022
DDx of Mediastinal Mass in Children
— Anterior
¡ Non-Hodgkin’s lymphoma
¡ Hodgkin’s disease
¡ Teratoma
— Middle
¡ Lymphoma
— Posterior
¡ Neuroblastoma
¡ Schannoma
178. Pediatric Board Review Hematology Oncology August 12, 2022
Question 35
— A 3 yr old girl who was known case of Acute Lymphoblasti
Leukemia (ALL Standard Risk) in Consolidation phase who was
brought by her parents to the emergency room with blurred
vision, difficulty in walking, headache, jaw pain and
numbness/tingling in fingers and toes. She is also constipated and
did not pass stools for the last 5 days. Which of the following
medications is most likely responsible for her symtpoms:
A. Doxorubicin
B. Methotrexate
C. Vincristine
D. PEG-Asparginase
E. Cytarabine
179. Pediatric Board Review Hematology Oncology August 12, 2022
Question 35
— A 3 yr old girl who was known case of Acute Lymphoblasti
Leukemia (ALL Standard Risk) in Consolidation phase who was
brought by her parents to the emergency room with blurred
vision, difficulty in walking, headache, jaw pain and
numbness/tingling in fingers and toes. She is also constipated and
did not pass stools for the last 5 days. Which of the following
medications is most likely responsible for her symtpoms:
A. Doxorubicin
B. Methotrexate
C. Vincristine
D. PEG-Asparginase
E. Cytarabine
180. Chemotherapy Agents
— Chemotherapy (also known as chemo) is a therapy in
which toxic drugs are given to the cancer patient to
interfere with the growth of the cancer cells.
— The goal of chemotherapy is to:
¡ Cure
¡ Control Disease
¡ Provide palliative care
Pediatric Board Review Hematology Oncology August 12, 2022
181. Chemotherapy Agents
Pediatric Board Review Hematology Oncology August 12, 2022
— Cell-Cycle Specific Agents:
¡ work by targeting the microtubules which form spindle fibers thus
interfering with cell division and resulting in cell death.
— Cell-Cycle Nonspecific Agents:
¡ damage the DNA by causing the DNA double-helix to break and/or
interfere with the DNA repair mechanism.