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Hematology - Oncology emergencies

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Interactive talk on common hematological and oncological emergencies - which if not noticed early can lead to irreversible complications and death .

Intended to be used for educational purposes for the fertile minds in medicine .

Published in: Health & Medicine
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Hematology - Oncology emergencies

  1. 1. Emergencies in Pediatric Hematology Akshat Jain MD, MPH’15 Division of Pediatrics , Pediatric Hematology Oncology and Stem Cell Transplantation Steven and Alexandra Cohen Children's Medical center of New York
  2. 2. Emergencies in Pediatric Hematology – Bleeding emergencies – Sickle cell emergencies – Anemia in a Neonate – Transfusion emergencies
  3. 3. Identifying abnormal “bleeding” • Epistaxis unrelieved by 15 minutes of pressure, both nostrils, requiring an ER visit, documented drop of Hb. • Bleeding after procedures (circumcision, dental extractions, T and A-delayed bleed) • Ecchymoses/bruising inconsistent with the degree of trauma. • Menstrual periods( amount, pads, duration)
  4. 4. Cause of bleeding in a child ! • Coagulation a. Primary hemostasis b. Secondary hemostasis • Vascular ( Non hematologic) Eg. Child Abuse ,Vasculitis
  5. 5. Causes of Bleeding - Coagulopathy: Congenital : • VWF Deficiency • Hemophilia • Platelet dysfunction Acquired : • DIC • Anticoagulants • Vit. K deficiency • Hepatic Failure • Renal Failure • Maternal Anticonvulsant
  6. 6. Characteristic Primary hemostasis Secondary hemostasis Onset Spontaneous and immediate Delayed after trauma Usual site Skin, mucous membranes Deep tissues / hemarthrosis Other sites Rare Retroperitoneum, CNS Examples Thrombocytopenia, platelet defects (vWD) Factor deficiency or inhibitor Case with Bleeding ? Think -
  7. 7. Case 1 : A healthy full term newborn has a completely normal physical exam except for a few petichiae. Platelet 50,000. THINK Production defects: Small platelets Genetic - TAR, Trisomy 13, 18 Wiskott- Aldrich Infections- viral, bacterial. Infiltration (Gauchers, Niemann Pick , Myelofibrosis , Osteopetrosis) ,Leukemia Destruction : Large platelets Allo-immune- Platelet group incompatibility. Auto-immune: Mat ITP Drugs (thiazide ,tolbutamide), SLE ,Infections Loss: Kasabach- Merritt syndrome (hemangiomas, DIC/TTP)
  8. 8. Kasabach-Merritt , TAR
  9. 9. Case 2 -Photo Quiz What’s different in this peripheral smear ? Normal Abnormal
  10. 10. ITP
  11. 11. ITP • Usually acute onset; immune mediated; post viral • Peak 2-5 years of age, males=females • Spontaneous bruises, petechiae • PE –no lymphadenopathy (LN), hepatosplenomegaly. • CBC- other cell lines normal, large platelets on smear • Treat if platelet < 10,000 or wet ITP, avoid NSAIDS, Aspirin. • Treat- IVIG best response, 48-72 hours; Side effects. – Anti-D (WInRho) Rh+ ,hemolysis, quick response – Steroids good response, SE, inexpensive, need BM • BM- Increased megakaryocytes, otherwise normal
  12. 12. Case 3 • 10 year old male treated with Valproic acid for seizures presents with fever. Well appreaing with no skin lesions, lymphadenopathy or hepatosplenomegaly. Labs - WBC 5K , Hb 12gm, Platelet 65,000. BUN 12, Creatinine 0.6 md/dl. • What is the Most likely cause: – ITP (Immune thrombocytopenic purpura) – HUS (Hemolytic Uremic Syndrome) – HS Purpura (Henoch- Schonlein Purpura) – ALL (Acute lymphoblastic leukemia) – Drug induced purpura
  13. 13. Bleeding Disorders Coagulation cascade
  14. 14. Bleeding Hemophiliac
  15. 15. The bleeding hemophiliac Hemophilia – – A (“classic”): factor 8 deficiency – B (“christmas disease”): factor 9 deficiency – Both coded on X chromosome – Disease due to absent or dysfunctional protein – Clinically INDISTINGUISHABLE – Prevalence: 1 in 7500 males
  16. 16. Basics of managing the bleeding hemophiliac • Rx - – Respective recombinant factor replacement (both F8 and F9 deficiency) – DDAVP (mild factor 8 deficiency) – Antifibrinolytics (amicar) • FUNDAMENTAL PRINCIPLE: – Recombinant factor 8 dosing: • 1 unit/kg of factor 8 raises the activity level 2% – Recombinant factor 9 dosing: • 1.5 unit/kg of r-factor 9 raises the acvity level 1% Life threatening Emergencies – FFP (factor 9 deficiency) – Cryoprecipitate (factor 8 deficiency)
  17. 17. Case 4 - What's Wrong ? 8 yo male with jaundice , Hgb- 8 gms , Elevated reticulocyte count Elevated total bilirubin , Abnormal cells on peripheral blood smear examination
  18. 18. Emergencies in Pediatric Hematology – Bleeding emergencies – Sickle cell emergencies – Anemia in a Neonate – Transfusion emergencies
  19. 19. Sickle cell emergencies
  20. 20. •Crises •Infection •Stroke •Sequestration •Acute chest syndrome
  21. 21. Sickle cell crises • Vasoocclusive crisis- dactylitis, long bones, back, chest. • Aplastic crisis – remember Parvovirus B19!
  22. 22. Sickle Cell Disease (SCD) • General principles : – Gentle hydration – Anti-inflammatory medications and narcotics . – STAT CBC w Reticulocyte count , Type and cross. – Supplemental oxygen ( controversial ) .
  23. 23. Can You Prevent a Pain Crisis? • Avoid extremes of temperatures; be prepared for sudden changes in temperature • Adequate hydration • Avoid swimming in cold water • Related to menstrual bleeding; consider contraception
  24. 24. Pain crisis Management • Warm, moist soaks , Never cold packs • Increase oral hydration • Ibuprofen • Opoids • Prefer not giving combinations preps – Percoset , Tylenon#3
  25. 25. Bacteremia and Sepsis Functional asplenia develops after repeated splenic infarctions in the 1st year of life. Leads to an increased risk of sepsis, particularly with Streptococcus pneumoniae. Prior to PPV 23, the risk of pneumococcal disease was 10% in children < 5years of age. 300 times greater than the general population.
  26. 26. PROPS I study Prophylaxis with Penicillin Protects Infants with SCA from Pneumococcal Sepsis Gaston et al. NEJM 1986 314:1593-159
  27. 27. Management of Fever • Fever is an emergency! • Do not use antipyretics for fever at home . • If temp >100.4 F (38.0 C) → ER visit . • Basic labs: CBC, U/A, cultures, CXR . • Empiric IV antibiotics (e.g., ceftriaxone 75 mg/kg) . • Observe for 3 hours . • Follow up in 24 hours – Give second IV antibiotic dose in 24 hours. • Admit if: toxic appearing, hemodynamic instability, WBC >30,000/µL or <5,000/µL, prior history of sepsis, temp > 40 C, social concerns . • Consider adding vancomycin if any of the above .
  28. 28. Stroke in SCD Occurs in 5 – 10% of children with HbSS Thrombotic or infarctive event involving large intracranial arteries Presents with weakness, aphasia, seizures, LOC Often results in permanent neurological damage and long- term disability
  29. 29. Rates of Stroke in HbSS by Age Ohene-Frempong et al Blood. 1998
  30. 30. STROKE • Can be ischemic (younger) or hemorrhagic (older ) . – Administer oxygen and IV fluids . – CBC w retic, Type and cross . – CT Scan brain . – Alert Blood bank STAT - exchange transfusion .
  31. 31. Secondary Prevention of Stroke  Without transfusion 70% recur within 3 years of initial stroke  Chronic transfusion therapy with an aim to keep HbS<30% at all times  Recurrence only 10% on chronic transfusion
  32. 32. Splenic Sequestration Blood can pool in spleen, causing hypovolemia. -I.V. Fluids, -Transfuse only to 8 or 9 g/Dl – NOT MORE
  33. 33. Spleenic sequestration
  34. 34. Case 5 • 12 year old female with SS disease complains of right sided chest pain and upper back pain for one day. • P/E reveals slightly reduced breath sounds and a Pulse OX of 86 %. CXR shows an infiltrate on the right lower lobe. • What is your diagnosis? • What will you do next?
  35. 35. ACS Gladwin MT, Vichinsky E. N Engl J Med 2008;359:2254-2265.
  36. 36. Definition- Diagnostic criteria for ACS • ACS is defined as radiographic evidence of consolidation: a new segmental radiographic pulmonary infiltrate, AND at least one of the following: 1. Temperature ≥38.5°C 2. >2 percent decrease in SpO2 (O2 saturation) from a documented steady-state value on room air (FiO2 = 0.21) 3. PaO2 <60 mmHg 4. Tachypnea (per age-adjusted normal) 5. Intercostal retractions, nasal flaring, or use of accessory muscles of respiration 6. Chest pain 7. Cough 8. Wheezing
  37. 37. Management of Acute Chest Syndrome • Antibiotics to cover pneumococcal, Mycoplasma, Chlamydia, • Bronchodilator, • Oxygen, • Incentive spirometry, • Transfusion, • Steroids (controversial), • NSAID’s • Avoid overhydration .
  38. 38. Prevention of ACS • Adequate pain control • Optimize pain control while minimizing sedation (PCA) • Incentive spirometry • Continuous pulse oximetry • Ambulation
  39. 39. Priapism • Caused by sickling of cells within the penis.  Results in a sustained erection  Painful; can lead to damage of penile tissues Mx. pointers – Don’t transfuse them.  Emergency evacuation of the hematoma rx of choice.  Oxygen  Vasodilators – pseudoephedrine
  40. 40. Pathologic Neonatal Anemia Think of : 1. Hemorrhage 2. Hemolysis 3. Inadequate Production
  41. 41. Work up
  42. 42. Case 6 • 1 week old male • Not Jaundiced • Hgb is 4 • Retic count is 0.5% What test do you want to order next?
  43. 43. Pathologic Anemia in Neonates • Can be divided into three categories: 1. Hemolysis 2. Inadequate Production 3. Hemorrhage
  44. 44. Differential Diagnosis
  45. 45. Case 6 • Low/Absent Reticulocyte count, think marrow dysfunction/failure and get a biopsy.
  46. 46. Inadequate Red Blood Cell Production • Can be caused by: 1. Lack of specific substrates or their carriers 2. Lack of an appropriate marrow environment 3. Lack of specific growth factors
  47. 47. Reticulocyte Count • If Low (0-2%) – Obtain Bone Marrow - Diamond-Blackfan - Fanconi’s Anemia - Congenital Dyserythropoetic Anemia - Refractory Sideroblastic Anemia - Transcobalamin II Deficiency - Other BMFS • If High (>5-10%) – Coomb’s Test
  48. 48. Case 7 • 1 week old female • Jaundiced • Hgb is 9 • Retic count is 7% What test do you want to order next? Direct Coombs test
  49. 49. Case 7 • Coomb’s is positive! So think - Isoimmunization…
  50. 50. Isoimmunization • ABO or Rh incompatibility is the most common cause of hemolytic disease in the newborn period • Other minor blood group incompatibilities include c, C, e, G, FGya (Duffy), Kell, Jka, MNS, Vw
  51. 51. • If Coombs is negative, Look at the Smear
  52. 52. Case 8 - • A 6 year old girl who has hereditary spherocytosis presents with a 1 week history of fever. Physical examination and history reveal abdominal pain, vomiting, fatigue and pallor. Her hemoglobin is typically about 10 g/dL with a reticulocyte count of 9%, but now, her hemoglobin is 4 g/dL and the reticulocyte count is 1%. Her bilirubin is 1 mg/dL. Of the following, the MOST likely cause for this girl’s present illness is infection with – Coxsackie virus – Epstein-Barr virus – Hepatitis A virus – Influenza A virus – Parvovirus B19
  53. 53. Hereditary Spherocytosis
  54. 54. Blood Smear • Specific RBC Dysmorphology - Elliptocytosis - Stomatocytes - Fragmentation - Basophilic Stipling - Spherocytes Obtain an osmotic fragility test
  55. 55. Hereditary Elliptocytosis
  56. 56. Case 9 • A previously normal African- American child visited Africa and was given malarial prophylaxis. He experienced pallor, fatigue, and dark urine. His hemoglobin level decreased from 14.8 to 9 g/dL. The most likely diagnosis is – Hereditary spherocytosis – Sickle cell disease – Hepatitis – G6PD deficiency • Blister cells • Bite cells • Blister cells • Bite cells
  57. 57. G6PD Deficiency • Acute Hemolytic Anemia is the most dramatic presentation of G6PD deficiency. • Acute intravascular hemolysis occurs after oxidative stress. • Stresses include: – Primaquine or sulfa drugs – Naphthalene (mothballs) – Fava beans – Infection
  58. 58. G6PD Deficiency Bite Cells When a macrophage in the spleen identifies an RBC with a Heinz body, it removes the precipitate and a small piece of the membrane Heinz Bodies Inclusions of denatured Hgb
  59. 59. • If no Jaundice – Acute Blood loss due to - Obstetric Complications - External Hemorrhage - Internal Hemorrhage - DIC/Sepsis - Bleeding Dyscrasias If no Jaundice…
  60. 60. Infections • Bacterial Infections • Parvovirus B19 • TORCH Infections • Syphilis • Malaria • HIV Get Blood Culture, Maternal Serum for IgG, HIV, RPR/FTA
  61. 61. Differential Diagnosis
  62. 62. Case 10 • 1 week old male • Jaundiced • Hgb is 9 • Retic count is 7% • Coomb’s is negative What test do you want to order next?
  63. 63. Case 10 • What does the smear show? • What tests do you want to order next?
  64. 64. Case 10 • Hypochromic, microcytic RBC’s, so think chronic bleed or thalessemia. • Obtain a Hgb electrophoresis and a Kleinhaur-Betke stain.
  65. 65. Case 11 • A 4 week old female Twin A, being seen in your clinic for anemia. • Born at 34 weeks, birth weight was 4lb 15oz. • An U/S 6 days prior to birth showed both babies to be moving well • Twin B was stillborn. Doctors told parents that Twin A had given blood to Twin B.
  66. 66. Pathologic Anemia in Neonates • Can be divided into three categories: 1. Inadequate Production 2. Hemolysis 3. Hemorrhage
  67. 67. Hemorrhage • Common cause of anemia in neonates • Can occur before birth or during delivery • Associated with obstetric accidents and maternal hemorrhage • Can also be due to internal hemorrhage or recurrent phlebotomy losses
  68. 68. Transfusion Medicine • Platelet transfusion – Platelet count < 10,000 – Active bleeding in setting of significant thrombocytopenia – Platelet count of 50,000 adequate for most surgical procedures – Single apheresis unit will generally raise platelet count by 30,000 • Anticipate low yield with splenomegaly, DIC, sepsis – Relative contraindications • ITP, TTP, and HIT – Indications of plasma transfusions • Coagulation factor deficiency – Consider factor concentrates if available • Rapid reversal of warfarin effect • Hemorrhage in patients with liver disease • DIC (controversial)
  69. 69. Transfusion Emergencies -Transfusion Reactions – Acute hemolytic reaction • Intravascular hemolysis (hemoglobinuria) – Fever, chills, pain, nausea, dyspnea, hypotension – May lead to ARF, DIC, and death • ABO incompatibility (human error) is most common cause – Proper labeling and patient identification is essential for prevention • STOP transfusion and notify blood bank immediately – Delayed hemolytic reaction • Occur days to weeks later • Caused by RBC antibodies not detected by pretransfusion testing – Febrile non-hemolytic reaction • Common in patients with multiple prior transfusions • Caused by cytokines or recipient antibodies to donor leukocytes
  70. 70. Transfusion Reactions  Hypotensive reaction  Caused by bradykinin generation  Hypotension and tachycardia shortly after beginning transfusion ; BP returns to baseline upon interruption  Usually safe to resume at slower rate upon recovery  Bacterial contamination  Septic shock ; high mortality  Rare but unpredictable  Transfusion-related acute lung injury (TRALI)  Occurs during or shortly after transfusion  Presents as pulmonary edema in absence of heart disease  Resolves within 48-72 hours  Mortality rate is 10%
  71. 71. I’m going to leave you with the following quiz that highlights the common and usually missed presentations of many oncologic diagnoses !!
  72. 72. Quiz 1 - What comes to mind with each of these presentations • Fever of unknown origin in a teenager with weight loss and itching ? • Infant less than 2 years of age with failure to thrive and irritability ? • Mass in the abdomen found by the babysitter?
  73. 73. Quiz 1 • Opsoclonus Myoclonus ? • Unilateral proptosis in childhood ? • Proptosis with black eyes ? • Bone pain and arthritis +/- neutropenia ?
  74. 74. Quiz 1 • Bone pain and swelling shortly after trauma ? • Bone Pain , weight loss and sick with normal blood counts ? • Pancytopenia and young red and white cell precursors on the blood smear ?
  75. 75. THANK YOU !

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