Mas
•Download as PPT, PDF•
4 likes•1,266 views
This patient presented with fever, rash, ankle pain, and respiratory distress. Testing showed elevated inflammatory markers, thrombocytopenia, and hyperferritinemia. A bone marrow biopsy showed hemophagocytosis, consistent with macrophage activation syndrome (MAS). Treatment with steroids and cyclosporine improved the patient's condition and laboratory abnormalities. The underlying diagnosis is likely a rheumatic condition presenting with MAS.
Report
Share
Report
Share
![Clinical History ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)
Recommended
Macrophage activation syndrome
Macrophage activation syndrome (MAS) is a life-threatening condition that can occur in patients with rheumatological disorders. It is characterized by proliferation and activation of macrophages that leads to cytokine overproduction and hemophagocytosis. The document discusses the pathogenesis, triggers, clinical features, diagnostic guidelines, management, and prognosis of MAS. Treatment involves high-dose corticosteroids as initial therapy, with cyclosporine A and other immunosuppressants used for severe or steroid-resistant cases. Early diagnosis and treatment have improved outcomes for MAS patients.
Haemophagocytic Syndrome
A 20-year-old woman presented with fever, fatigue, and weight loss. She had a history of Holt-Oram syndrome and primary EBV infection a year prior. Testing showed pancytopenia, hepatosplenomegaly, and elevated ferritin. A bone marrow biopsy showed hemophagocytosis. She was diagnosed with EBV-associated hemophagocytic syndrome and died despite treatment. Hemophagocytic syndrome is caused by impaired NK and cytotoxic T cell function leading to uncontrolled immune activation and cellular damage. It can be genetic, infection-associated, malignancy-associated, or associated with autoimmune diseases. Treatment involves controlling inflammation, the underlying trigger, and supportive care.
Hemophagocytic lymphohistiocytosis (HLH)
Hemophagocytic Lymphohistiocytosis (HLH) is an aggressive and life threatening syndrome which results from excessive immune activation, that can rapidly deteriorate and lead to multiple organ failure and death.
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis - definition, etiology, pathophysiology, primary and secondary HLH, clinical findings, investigations, diagnostic criteria, treatment
Hemophagocytic syndrome.
This document presents two case reports of hemophagocytic syndrome (HPS). HPS is a rare disorder characterized by uncontrolled phagocytosis of blood cells in the bone marrow. It can be secondary to infections, malignancies, autoimmune diseases, or immunodeficiencies. The first case report describes an 11-month old child with HPS associated with Plasmodium falciparum malaria infection. The second case report involves a 17-year old male with HPS complicating tuberculosis infection. Both patients exhibited cytopenias, hepatosplenomegaly, and hemophagocytosis identified in bone marrow samples. The malaria patient recovered with antimalarial treatment, while the tuberculosis patient responded to antitub
Hemophagocytic Lymphohistiocytosis - HLH
Hemophagocytic Lymphohistiocytosis - HLH
epidemiology, clinical presentation, treatments and outcomes.
Hl hing
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by uncontrolled activation of macrophages and lymphocytes, leading to excessive inflammation and organ damage. It can occur primarily due to genetic mutations or secondarily in association with infections, malignancies, or rheumatologic conditions. The pathophysiology involves defective cytotoxic lymphocyte function and cytokine overproduction. HLH is diagnosed based on clinical and laboratory criteria including prolonged fever, cytopenias, hypertriglyceridemia/hypofibrinogenemia, hyperferritinemia, and hemophagocytosis. Early diagnosis and treatment with chemotherapy, steroids, and hematopoietic stem cell transplantation is needed for survival, as H
Admissions from Cardiac Cath Lab to RICU
This document presents a case report of a 41-year-old man admitted with fever, respiratory symptoms, and deranged liver function tests. Further investigations revealed pancytopenia, hyperferritinemia over 11,000 ng/ml, and bone marrow biopsy showing hemophagocytosis, leading to a diagnosis of acquired hemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus infection. HLH is a rare but under-recognized hyperinflammatory condition that can cause multi-organ dysfunction resembling sepsis. It requires specific treatment including dexamethasone, cyclosporine A, and etoposide to control the uncontrolled activation of macrophages and lymphocytes driving the hyperinflammatory state. The
Recommended
Macrophage activation syndrome
Macrophage activation syndrome (MAS) is a life-threatening condition that can occur in patients with rheumatological disorders. It is characterized by proliferation and activation of macrophages that leads to cytokine overproduction and hemophagocytosis. The document discusses the pathogenesis, triggers, clinical features, diagnostic guidelines, management, and prognosis of MAS. Treatment involves high-dose corticosteroids as initial therapy, with cyclosporine A and other immunosuppressants used for severe or steroid-resistant cases. Early diagnosis and treatment have improved outcomes for MAS patients.
Haemophagocytic Syndrome
A 20-year-old woman presented with fever, fatigue, and weight loss. She had a history of Holt-Oram syndrome and primary EBV infection a year prior. Testing showed pancytopenia, hepatosplenomegaly, and elevated ferritin. A bone marrow biopsy showed hemophagocytosis. She was diagnosed with EBV-associated hemophagocytic syndrome and died despite treatment. Hemophagocytic syndrome is caused by impaired NK and cytotoxic T cell function leading to uncontrolled immune activation and cellular damage. It can be genetic, infection-associated, malignancy-associated, or associated with autoimmune diseases. Treatment involves controlling inflammation, the underlying trigger, and supportive care.
Hemophagocytic lymphohistiocytosis (HLH)
Hemophagocytic Lymphohistiocytosis (HLH) is an aggressive and life threatening syndrome which results from excessive immune activation, that can rapidly deteriorate and lead to multiple organ failure and death.
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis - definition, etiology, pathophysiology, primary and secondary HLH, clinical findings, investigations, diagnostic criteria, treatment
Hemophagocytic syndrome.
This document presents two case reports of hemophagocytic syndrome (HPS). HPS is a rare disorder characterized by uncontrolled phagocytosis of blood cells in the bone marrow. It can be secondary to infections, malignancies, autoimmune diseases, or immunodeficiencies. The first case report describes an 11-month old child with HPS associated with Plasmodium falciparum malaria infection. The second case report involves a 17-year old male with HPS complicating tuberculosis infection. Both patients exhibited cytopenias, hepatosplenomegaly, and hemophagocytosis identified in bone marrow samples. The malaria patient recovered with antimalarial treatment, while the tuberculosis patient responded to antitub
Hemophagocytic Lymphohistiocytosis - HLH
Hemophagocytic Lymphohistiocytosis - HLH
epidemiology, clinical presentation, treatments and outcomes.
Hl hing
Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by uncontrolled activation of macrophages and lymphocytes, leading to excessive inflammation and organ damage. It can occur primarily due to genetic mutations or secondarily in association with infections, malignancies, or rheumatologic conditions. The pathophysiology involves defective cytotoxic lymphocyte function and cytokine overproduction. HLH is diagnosed based on clinical and laboratory criteria including prolonged fever, cytopenias, hypertriglyceridemia/hypofibrinogenemia, hyperferritinemia, and hemophagocytosis. Early diagnosis and treatment with chemotherapy, steroids, and hematopoietic stem cell transplantation is needed for survival, as H
Admissions from Cardiac Cath Lab to RICU
This document presents a case report of a 41-year-old man admitted with fever, respiratory symptoms, and deranged liver function tests. Further investigations revealed pancytopenia, hyperferritinemia over 11,000 ng/ml, and bone marrow biopsy showing hemophagocytosis, leading to a diagnosis of acquired hemophagocytic lymphohistiocytosis (HLH) secondary to Epstein-Barr virus infection. HLH is a rare but under-recognized hyperinflammatory condition that can cause multi-organ dysfunction resembling sepsis. It requires specific treatment including dexamethasone, cyclosporine A, and etoposide to control the uncontrolled activation of macrophages and lymphocytes driving the hyperinflammatory state. The
Hemophagocytic lymphohistiocytosis hlh 2019
Hemophagocytic lymphohistiocytosis
hlh presentation
Hemophagocytic lymphohistiocytosis presentation
hlh
Hemophagocyitic histiocytosis
This document presents a case of a 4-year-old boy with 1 month of intermittent high fever and pallor who was found to have severe anemia, jaundice, hepatosplenomegaly, and bone marrow findings consistent with hemophagocytic lymphohistiocytosis (HLH). HLH is a life-threatening syndrome characterized by uncontrolled activation of macrophages and T-cells leading to hyperinflammation. It can be primary/familial or secondary to infections, malignancies, or autoimmune conditions. The case report reviews the diagnostic criteria, associated infections, clinical manifestations, bone marrow findings, treatment including chemotherapy and stem cell transplant, and prognosis of HLH.
Hemophagocytic lymphohistocytosis Awareness HLH
A 21-year-old man presented with 10 days of fever, headache, and jaundice. On examination, he had a temperature of 40.7°C, jaundice, and enlarged spleen and liver. Laboratory tests showed pancytopenia, elevated liver enzymes and ferritin. Further tests ruled out infections but HLH criteria were met with ferritin >40000 and sCD25 >7500. A bone marrow biopsy showed hemophagocytosis. The patient was diagnosed with secondary HLH and treated with HLH-94 protocol chemotherapy including etoposide and dexamethasone, with consideration for hematopoietic stem cell transplant if the initial treatment is not effective.
Kawasaki with hlh,an unusual case of fever
This document describes the case of a 1-year-old boy who presented with prolonged fever for 10 days. Investigations revealed hepatosplenomegaly, anemia, thrombocytopenia, and elevated inflammatory markers. Echocardiography showed a right coronary artery aneurysm, suggestive of atypical Kawasaki disease. Bone marrow examination showed hemophagocytosis. The patient was diagnosed with atypical Kawasaki disease complicated by secondary hemophagocytic lymphohistiocytosis (HLH). Treatment with intravenous immunoglobulin and aspirin led to improvement of symptoms and regression of organomegaly within a week. Follow up showed no abnormalities. The document discusses that 1.9% of Kawasaki
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH) is not an uncommon disorder. It should be actively considered when we have an acutely ill child with fever, organomegaly, rapidly evolving cytopenias and deranged liver functions. It is a life-threatening disease characterized by uncontrolled hyperinflammation on the basis of a variety of inherited or acquired immune deficiency. Paediatricians, especially in the tertiary care setting, need to be sensitized about its clinical symptoms and diagnostic criteria so that we can offer timely treatment. This article characterizes this condition in detail and outlines its treatment.
HLHresearchweb
1) HLH is a rare and deadly disease with room for improvement in diagnosis, initial therapies, and bone marrow transplantation.
2) The development of a mouse model of HLH has helped researchers understand the immunopathology and identify rational treatment targets.
3) A new clinical trial is combining ATG and etoposide therapies and exploring newer BMT strategies to improve survival in HLH patients.
4) Promising research is also investigating gene therapy approaches to correct the underlying genetic defects causing HLH.
Another Day, Another Fever
Gigi Blanchard, MD, from UC San Diego Owen Clinic, presents "Another Day, Another Fever" at AIDS Clinical Rounds
Macrophage activation
This document discusses the case of a 63-year-old woman with rheumatoid arthritis and diabetes who presented with chest pain and was diagnosed with a myocardial infarction. She subsequently developed fever and pancytopenia. Further tests found very high ferritin levels and a bone marrow biopsy confirmed macrophage activation syndrome, a severe complication caused by overly active immune cells. She was started on treatment including steroids and IVIG but unfortunately died shortly after being transferred to the ICU. The key lessons are that macrophage activation syndrome can occur in adults with autoimmune conditions and be fatal if not recognized and treated promptly.
A Case of Pure Red Cell Aplasia
The document describes a case of a 42-year-old man presenting with breathlessness and anemia who was diagnosed with a mediastinal mass (thymoma) and pure red cell aplasia (PRCA) based on bone marrow findings. He underwent thymectomy and was treated with steroids for his PRCA. PRCA is a rare cause of normocytic normochromic anemia characterized by absence of erythroblasts in the bone marrow that can be associated with thymoma and treated with steroids, IVIG, or surgery.
PRCA post renal transplant-a case and review
Partha Choudhary, a 48-year-old male, developed progressive anemia after a liver transplant in 2011. His hemoglobin levels gradually decreased from 9.2 to 5.4 over a year despite EPO treatment. Testing found he was positive for Parvovirus B19 and negative for other potential causes. A bone marrow biopsy showed an absence of erythroblasts, consistent with a diagnosis of pure red cell aplasia caused by Parvovirus B19 infection. Treatment for this type of virus-induced PRCA typically involves intravenous immunoglobulin therapy.
Thrombotic Microangiopathy associated with proteasome inhibitors
This case report describes a 63-year-old man who developed acute kidney injury and thrombotic microangiopathy after receiving treatment with the proteasome inhibitor carfilzomib. A kidney biopsy showed features of thrombotic microangiopathy and arteriolar damage. The authors propose that proteasome inhibitors may cause kidney injury by decreasing VEGF production through inhibition of the NF-κB pathway, disrupting normal endothelial cell function and integrity. The patient's kidney function recovered after discontinuing carfilzomib with supportive management. This case adds to evidence that proteasome inhibitors can cause thrombotic microangiopathy through their effects on VEGF signaling.
Anti gbm
Anti-GBM disease is a rare autoimmune disease characterized by antibodies against type IV collagen in the basement membrane of the glomeruli and lungs. It commonly presents as rapidly progressive glomerulonephritis and pulmonary hemorrhage. Treatment involves plasma exchange to remove antibodies combined with cyclophosphamide and corticosteroids to suppress antibody production. Prognosis depends on renal function at presentation, with most patients achieving remission if treated early before requiring dialysis. Relapse is rare but can occur if exposures like smoking continue.
Thrombotic thrombocytopenic purpura in pregnancy as grave as it comes jiacm...
This case report describes an 18-year-old pregnant woman diagnosed with thrombotic thrombocytopenic purpura (TTP) at 26 weeks gestation. She presented with fever, headache, seizures, and was found to have microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. She underwent an emergency delivery of a stillborn infant due to severe preeclampsia. MRI revealed features of posterior reversible encephalopathy syndrome (PRES). Plasma exchange therapy was initiated and her symptoms improved. TTP is a rare but life-threatening condition in pregnancy that requires prompt diagnosis and treatment such as plasma exchange to improve outcomes.
MAHA/TTP/DIC/HUS/aHUS
This document discusses microangiopathic hemolytic anemia (MAHA) and thrombotic thrombocytopenic purpura (TTP). It recommends ordering tests like LDH, haptoglobin, reticulocyte count, and ADAMTS13 level if MAHA is suspected. ADAMTS13 levels below 10% are specific for TTP, caused by acquired or hereditary deficiencies of the von Willebrand factor cleaving protease ADAMTS13. Initial treatment for suspected TTP includes plasma exchange while waiting for test results. Differential diagnoses are discussed including thrombotic microangiopathies, autoimmune hemolytic anemia, immune thrombocytopenic purpura, and aplastic
Transplantation associated Thrombotic microangiopathy (TA-TMA)
Bone marrow transplantation associated Thrombotic Microangiopathy - history, definition, epidemiology, risk factors, pathogenesis, clinical features, lab studies, diagnostic criteria, treatment, prognosis, differences from Idiopathic TTP
MSKCC TMA Lecture
This document discusses a case of thrombotic microangiopathy (TMA) occurring in a 24-year-old pregnant female who presented with fever, fatigue, and abdominal pain. She was found to have sepsis and disseminated intravascular coagulation (DIC) and was treated with antibiotics, fluids and pressors. Her condition deteriorated and she developed renal failure. Testing found low levels of ADAMTS13 and a diagnosis of atypical hemolytic uremic syndrome (aHUS) was made. She was treated with plasma exchange, hemodialysis and the complement inhibitor eculizumab, which led to improvement in her hematologic and renal parameters and eventual discharge.
Takaysu Glomerulonephritis
This document discusses two cases of Takayasu arteritis (TA). The first case involves a 17-year-old female presenting with constitutional symptoms and renal dysfunction who was found to have left subclavian artery stenosis on imaging. The second case discusses the rare association between TA and glomerular disease, including reports of membranoproliferative glomerulonephritis and IgA nephropathy in patients with TA. Treatment of the renal manifestations of TA typically involves corticosteroids and immunosuppression.
Refractory ITP
Slide ประกอบการบรรยายเรื่อง refractory ITP โดย รศ.พลภัทร โรจน์นครินทร์ ในการประชุมกลางปี สมาคมโลหิตวิทยาแห่งประเทศไทย พฤศจิกายน 2552
Tma lecture mskcc
The document discusses thrombotic microangiopathies (TMAs) such as thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS). TMAs are caused by endothelial cell damage and platelet activation leading to thrombosis. While plasma therapy can induce remission in some patients with aHUS, genetic mutations affecting complement regulatory factors often result in poor long-term outcomes of end-stage renal disease or death despite treatment.
Case presentation
A 23-year old female presents with a rash, bruising, nosebleeds, and heavy menstruation. Her physical exam and labs reveal an isolated thrombocytopenia. Her peripheral smear shows decreased platelet numbers and slightly larger platelets, suggesting early release from the bone marrow in response to peripheral destruction. Further history and testing are needed to determine the cause of the thrombocytopenia.
Adult Onset Still's Disease
Adult-onset Still's disease is a form of rheumatoid arthritis that was characterized by Bywaters in 1971. Its main feature is a combination of symptoms such as high fever, cutaneous rash during fever peaks, joint and muscle pain, lymph node enlargement, increased white cell count especially polymorphonuclear neutrophils and abnormalities of liver metabolism. None of these symptoms is sufficient to establish the diagnosis and infact may be present in several other diseases such as neoplastic and infectious diseases. Thus AOSD is a “diagnosis of exclusion”.
24 glomerular disease
The document provides an overview of glomerular disease, including anatomy and function of the kidney and nephron, general pathogenesis, clinical syndromes and presentations, and specific glomerular diseases. It discusses topics like minimal change disease, membranous nephropathy, nephrotic syndrome, acute glomerulonephritis, rapidly progressive glomerulonephritis, and chronic glomerulonephritis. Treatment approaches for various conditions are also summarized.
More Related Content
What's hot
Hemophagocytic lymphohistiocytosis hlh 2019
Hemophagocytic lymphohistiocytosis
hlh presentation
Hemophagocytic lymphohistiocytosis presentation
hlh
Hemophagocyitic histiocytosis
This document presents a case of a 4-year-old boy with 1 month of intermittent high fever and pallor who was found to have severe anemia, jaundice, hepatosplenomegaly, and bone marrow findings consistent with hemophagocytic lymphohistiocytosis (HLH). HLH is a life-threatening syndrome characterized by uncontrolled activation of macrophages and T-cells leading to hyperinflammation. It can be primary/familial or secondary to infections, malignancies, or autoimmune conditions. The case report reviews the diagnostic criteria, associated infections, clinical manifestations, bone marrow findings, treatment including chemotherapy and stem cell transplant, and prognosis of HLH.
Hemophagocytic lymphohistocytosis Awareness HLH
A 21-year-old man presented with 10 days of fever, headache, and jaundice. On examination, he had a temperature of 40.7°C, jaundice, and enlarged spleen and liver. Laboratory tests showed pancytopenia, elevated liver enzymes and ferritin. Further tests ruled out infections but HLH criteria were met with ferritin >40000 and sCD25 >7500. A bone marrow biopsy showed hemophagocytosis. The patient was diagnosed with secondary HLH and treated with HLH-94 protocol chemotherapy including etoposide and dexamethasone, with consideration for hematopoietic stem cell transplant if the initial treatment is not effective.
Kawasaki with hlh,an unusual case of fever
This document describes the case of a 1-year-old boy who presented with prolonged fever for 10 days. Investigations revealed hepatosplenomegaly, anemia, thrombocytopenia, and elevated inflammatory markers. Echocardiography showed a right coronary artery aneurysm, suggestive of atypical Kawasaki disease. Bone marrow examination showed hemophagocytosis. The patient was diagnosed with atypical Kawasaki disease complicated by secondary hemophagocytic lymphohistiocytosis (HLH). Treatment with intravenous immunoglobulin and aspirin led to improvement of symptoms and regression of organomegaly within a week. Follow up showed no abnormalities. The document discusses that 1.9% of Kawasaki
Hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis (HLH) is not an uncommon disorder. It should be actively considered when we have an acutely ill child with fever, organomegaly, rapidly evolving cytopenias and deranged liver functions. It is a life-threatening disease characterized by uncontrolled hyperinflammation on the basis of a variety of inherited or acquired immune deficiency. Paediatricians, especially in the tertiary care setting, need to be sensitized about its clinical symptoms and diagnostic criteria so that we can offer timely treatment. This article characterizes this condition in detail and outlines its treatment.
HLHresearchweb
1) HLH is a rare and deadly disease with room for improvement in diagnosis, initial therapies, and bone marrow transplantation.
2) The development of a mouse model of HLH has helped researchers understand the immunopathology and identify rational treatment targets.
3) A new clinical trial is combining ATG and etoposide therapies and exploring newer BMT strategies to improve survival in HLH patients.
4) Promising research is also investigating gene therapy approaches to correct the underlying genetic defects causing HLH.
Another Day, Another Fever
Gigi Blanchard, MD, from UC San Diego Owen Clinic, presents "Another Day, Another Fever" at AIDS Clinical Rounds
Macrophage activation
This document discusses the case of a 63-year-old woman with rheumatoid arthritis and diabetes who presented with chest pain and was diagnosed with a myocardial infarction. She subsequently developed fever and pancytopenia. Further tests found very high ferritin levels and a bone marrow biopsy confirmed macrophage activation syndrome, a severe complication caused by overly active immune cells. She was started on treatment including steroids and IVIG but unfortunately died shortly after being transferred to the ICU. The key lessons are that macrophage activation syndrome can occur in adults with autoimmune conditions and be fatal if not recognized and treated promptly.
A Case of Pure Red Cell Aplasia
The document describes a case of a 42-year-old man presenting with breathlessness and anemia who was diagnosed with a mediastinal mass (thymoma) and pure red cell aplasia (PRCA) based on bone marrow findings. He underwent thymectomy and was treated with steroids for his PRCA. PRCA is a rare cause of normocytic normochromic anemia characterized by absence of erythroblasts in the bone marrow that can be associated with thymoma and treated with steroids, IVIG, or surgery.
PRCA post renal transplant-a case and review
Partha Choudhary, a 48-year-old male, developed progressive anemia after a liver transplant in 2011. His hemoglobin levels gradually decreased from 9.2 to 5.4 over a year despite EPO treatment. Testing found he was positive for Parvovirus B19 and negative for other potential causes. A bone marrow biopsy showed an absence of erythroblasts, consistent with a diagnosis of pure red cell aplasia caused by Parvovirus B19 infection. Treatment for this type of virus-induced PRCA typically involves intravenous immunoglobulin therapy.
Thrombotic Microangiopathy associated with proteasome inhibitors
This case report describes a 63-year-old man who developed acute kidney injury and thrombotic microangiopathy after receiving treatment with the proteasome inhibitor carfilzomib. A kidney biopsy showed features of thrombotic microangiopathy and arteriolar damage. The authors propose that proteasome inhibitors may cause kidney injury by decreasing VEGF production through inhibition of the NF-κB pathway, disrupting normal endothelial cell function and integrity. The patient's kidney function recovered after discontinuing carfilzomib with supportive management. This case adds to evidence that proteasome inhibitors can cause thrombotic microangiopathy through their effects on VEGF signaling.
Anti gbm
Anti-GBM disease is a rare autoimmune disease characterized by antibodies against type IV collagen in the basement membrane of the glomeruli and lungs. It commonly presents as rapidly progressive glomerulonephritis and pulmonary hemorrhage. Treatment involves plasma exchange to remove antibodies combined with cyclophosphamide and corticosteroids to suppress antibody production. Prognosis depends on renal function at presentation, with most patients achieving remission if treated early before requiring dialysis. Relapse is rare but can occur if exposures like smoking continue.
Thrombotic thrombocytopenic purpura in pregnancy as grave as it comes jiacm...
This case report describes an 18-year-old pregnant woman diagnosed with thrombotic thrombocytopenic purpura (TTP) at 26 weeks gestation. She presented with fever, headache, seizures, and was found to have microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. She underwent an emergency delivery of a stillborn infant due to severe preeclampsia. MRI revealed features of posterior reversible encephalopathy syndrome (PRES). Plasma exchange therapy was initiated and her symptoms improved. TTP is a rare but life-threatening condition in pregnancy that requires prompt diagnosis and treatment such as plasma exchange to improve outcomes.
MAHA/TTP/DIC/HUS/aHUS
This document discusses microangiopathic hemolytic anemia (MAHA) and thrombotic thrombocytopenic purpura (TTP). It recommends ordering tests like LDH, haptoglobin, reticulocyte count, and ADAMTS13 level if MAHA is suspected. ADAMTS13 levels below 10% are specific for TTP, caused by acquired or hereditary deficiencies of the von Willebrand factor cleaving protease ADAMTS13. Initial treatment for suspected TTP includes plasma exchange while waiting for test results. Differential diagnoses are discussed including thrombotic microangiopathies, autoimmune hemolytic anemia, immune thrombocytopenic purpura, and aplastic
Transplantation associated Thrombotic microangiopathy (TA-TMA)
Bone marrow transplantation associated Thrombotic Microangiopathy - history, definition, epidemiology, risk factors, pathogenesis, clinical features, lab studies, diagnostic criteria, treatment, prognosis, differences from Idiopathic TTP
MSKCC TMA Lecture
This document discusses a case of thrombotic microangiopathy (TMA) occurring in a 24-year-old pregnant female who presented with fever, fatigue, and abdominal pain. She was found to have sepsis and disseminated intravascular coagulation (DIC) and was treated with antibiotics, fluids and pressors. Her condition deteriorated and she developed renal failure. Testing found low levels of ADAMTS13 and a diagnosis of atypical hemolytic uremic syndrome (aHUS) was made. She was treated with plasma exchange, hemodialysis and the complement inhibitor eculizumab, which led to improvement in her hematologic and renal parameters and eventual discharge.
Takaysu Glomerulonephritis
This document discusses two cases of Takayasu arteritis (TA). The first case involves a 17-year-old female presenting with constitutional symptoms and renal dysfunction who was found to have left subclavian artery stenosis on imaging. The second case discusses the rare association between TA and glomerular disease, including reports of membranoproliferative glomerulonephritis and IgA nephropathy in patients with TA. Treatment of the renal manifestations of TA typically involves corticosteroids and immunosuppression.
Refractory ITP
Slide ประกอบการบรรยายเรื่อง refractory ITP โดย รศ.พลภัทร โรจน์นครินทร์ ในการประชุมกลางปี สมาคมโลหิตวิทยาแห่งประเทศไทย พฤศจิกายน 2552
Tma lecture mskcc
The document discusses thrombotic microangiopathies (TMAs) such as thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS). TMAs are caused by endothelial cell damage and platelet activation leading to thrombosis. While plasma therapy can induce remission in some patients with aHUS, genetic mutations affecting complement regulatory factors often result in poor long-term outcomes of end-stage renal disease or death despite treatment.
Case presentation
A 23-year old female presents with a rash, bruising, nosebleeds, and heavy menstruation. Her physical exam and labs reveal an isolated thrombocytopenia. Her peripheral smear shows decreased platelet numbers and slightly larger platelets, suggesting early release from the bone marrow in response to peripheral destruction. Further history and testing are needed to determine the cause of the thrombocytopenia.
What's hot (20)
Thrombotic Microangiopathy associated with proteasome inhibitors
Thrombotic Microangiopathy associated with proteasome inhibitors
Thrombotic thrombocytopenic purpura in pregnancy as grave as it comes jiacm...
Thrombotic thrombocytopenic purpura in pregnancy as grave as it comes jiacm...
Transplantation associated Thrombotic microangiopathy (TA-TMA)
Transplantation associated Thrombotic microangiopathy (TA-TMA)
Similar to Mas
Adult Onset Still's Disease
Adult-onset Still's disease is a form of rheumatoid arthritis that was characterized by Bywaters in 1971. Its main feature is a combination of symptoms such as high fever, cutaneous rash during fever peaks, joint and muscle pain, lymph node enlargement, increased white cell count especially polymorphonuclear neutrophils and abnormalities of liver metabolism. None of these symptoms is sufficient to establish the diagnosis and infact may be present in several other diseases such as neoplastic and infectious diseases. Thus AOSD is a “diagnosis of exclusion”.
24 glomerular disease
The document provides an overview of glomerular disease, including anatomy and function of the kidney and nephron, general pathogenesis, clinical syndromes and presentations, and specific glomerular diseases. It discusses topics like minimal change disease, membranous nephropathy, nephrotic syndrome, acute glomerulonephritis, rapidly progressive glomerulonephritis, and chronic glomerulonephritis. Treatment approaches for various conditions are also summarized.
A Case of Hepato-Pulmonary Syndrome
This case involves a 35-year-old female with a history of non-cirrhotic portal fibrosis, splenectomy, recurrent tuberculosis, and recurrent bouts of hematemesis and fever. She presents with cyanosis, clubbing, and shortness of breath. Testing reveals findings consistent with hepatopulmonary syndrome, including low oxygen saturation that decreases further in an upright position. A contrast echocardiogram confirms intrapulmonary vascular dilations. She is diagnosed with severe, longstanding hepatopulmonary syndrome secondary to non-cirrhotic portal hypertension.
Prolonged fever in a 12 year old girl
This document presents a case study of a 12-year-old girl who presented with prolonged fever for over 20 days. Testing showed bi-cytopenia (anemia and leukopenia), hepatosplenomegaly, lymphadenopathy, and positive EBV and parvovirus serology. A bone marrow biopsy revealed hemophagocytosis. She was ultimately diagnosed with hemophagocytic lymphohistiocytosis (HLH), a life-threatening hyperinflammatory condition. Treatment for HLH involves prompt institution of therapies like steroids, chemotherapy, to prevent irreversible organ damage from the hyperactive immune response.
Calcifilaxia iii
This case report describes a 61-year-old woman who developed calcific uraemic arteriolopathy (CUA), a condition characterized by small vessel calcification and occlusion leading to skin lesions and ulcers. She had multiple risk factors for CUA including end-stage renal failure, hyperparathyroidism, steroid use, and warfarin treatment. Surgical biopsy confirmed CUA as the cause of her non-healing leg ulcers. CUA has a high mortality rate due to secondary infection but treatment aims to control calcium and phosphate levels, optimize wound care, and improve tissue oxygen delivery. The pathogenesis of CUA involves abnormalities in calcium-phosphate metabolism and potential deficiencies in natural inhibitors of vascular calcification
Multiple Myeloma (Case presentation)
This case presentation describes a 55-year-old male referred for management of severe anemia. He has a history of recurrent kidney stones, hypertension, diabetes, and back pain. Laboratory tests reveal severe anemia with rouleaux formation. Further workup shows evidence of a gammopathy. A bone marrow biopsy is needed to confirm a diagnosis of multiple myeloma, which can cause anemia and kidney damage through paraprotein production and bone lesions. Treatment involves supportive care, chemotherapy, and stem cell transplantation in eligible patients.
Junior Medillectuals- Prelims
The document discusses two conditions: paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS). PNH is a complement-mediated hemolytic anemia treated with the drug eculizumab, a C5a inhibitor. aHUS is a complement-mediated thrombotic microangiopathy that can cause stroke, heart attack, and kidney failure, and is also treated with eculizumab. The document asks the reader to identify PNH, aHUS, and the mode of action of eculizumab.
A Case of Seronegative Autoimmune Hepatitis
IOSR Journal of Dental and Medical Sciences is one of the speciality Journal in Dental Science and Medical Science published by International Organization of Scientific Research (IOSR). The Journal publishes papers of the highest scientific merit and widest possible scope work in all areas related to medical and dental science. The Journal welcome review articles, leading medical and clinical research articles, technical notes, case reports and others.
Expression of AFP, P-sel, and MMP-9 in Cirrhosis with Portal Vein Thrombosis
Expression of AFP, P-sel, and MMP-9 in Cirrhosis with Portal Vein ThrombosisANALYTICAL AND QUANTITATIVE CYTOPATHOLOGY AND HISTOPATHOLOGY
This study analyzed the expression and clinical significance of serum biomarkers AFP, P-selectin (P-sel), and MMP-9 in patients with hepatic sclerosis combined with portal vein thrombosis (PVT). The study found that levels of all three biomarkers were significantly higher in patients with hepatic sclerosis and PVT compared to patients with hepatic sclerosis alone. Furthermore, AFP, P-sel, and MMP-9 were identified as main risk factors for PVT. The expression of AFP was also found to be positively correlated with the expression of P-sel and MMP-9 in patients with hepatic sclerosis and PVT.Autoimmune Hepatitis
Overview of Autoimmune Hepatitis, overlap syndromes or variants. Disease ovedrview, workup, diagnosis, management and recent advances.
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
This case report describes a patient with a mild form of familial Mediterranean fever (FMF) associated with a polymorphism in the MEFV gene. The patient experienced recurrent fever episodes accompanied by mild skin rashes and joint pain. Genetic testing identified a c.1588-69G>A polymorphism in intron 5 of the MEFV gene. The patient responded well to treatment with corticosteroids and colchicine, though had to discontinue colchicine due to side effects. The polymorphism is not rare and has been found in healthy subjects, suggesting it is associated with a milder form of FMF that requires other triggers to induce symptoms.
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease caused bymutation(s) in the Mediterranean fever (MEFV, pyrinmarenostrin) gene. FMF is characterized byrecurrent fever crisis combined with serosal, synovial, or cutìaneous inflammation. Until now morethan 304 sequence variants have been recorded. Here, we describe a case of mild FMF confirmedby...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
This case report describes a patient with a mild form of familial Mediterranean fever (FMF) associated with a polymorphism in the MEFV gene. The patient experienced recurrent fever episodes accompanied by mild skin rashes and joint pain. Genetic testing identified a c.1588-69G>A polymorphism in intron 5 of the MEFV gene. The patient responded well to treatment with corticosteroids and colchicine, though had to discontinue colchicine due to side effects. The polymorphism is not rare and has been found in healthy individuals, suggesting it is associated with a milder form of FMF that requires other triggers to induce symptoms.
Thrombosis Case Study
A 27-year-old woman presented with fatigue, shortness of breath, easy bruising, and a syncopal episode. Laboratory workup revealed hemolytic anemia. A bone marrow biopsy showed hypocellular marrow consistent with aplastic anemia and paroxysmal nocturnal hemoglobinuria (PNH). She was treated with immunosuppression but later developed multiple thromboses. Testing confirmed PNH diagnosis. She received eculizumab therapy which stabilized her hemoglobin levels and reduced transfusion requirements. PNH causes hemolytic anemia due to lack of protective proteins on blood cells, predisposing to complement-mediated destruction and thrombosis.
Nephrotic And Nephritic Syndrome 2008
This document defines and compares nephrotic syndrome and nephritic syndrome. It provides details on various causes of glomerular disease including minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA nephropathy, lupus nephritis, anti-GBM disease, and rapidly progressive glomerulonephritis. Evaluation of glomerular disease involves history, exam, urinalysis, and complement levels with renal biopsy used to confirm specific diagnoses.
Hypereosinophilia overview - pediatric resident
Hypereosinophilia overview
by a pediatric resident
immunology and allergy
Shaikah Abdulla Bawazir
Antiphospholipid antibody syndrome
This document summarizes antiphospholipid antibody syndrome (APLS). Key points:
- APLS is an autoimmune condition characterized by vascular thrombosis and/or pregnancy morbidity associated with persistent antiphospholipid antibodies.
- It is also known as Hughes syndrome. Common clinical manifestations include venous thromboembolism, arterial thrombosis, recurrent miscarriages, and cardiac valve disease.
- The pathogenesis involves antiphospholipid antibodies interfering with coagulation pathways and activating endothelial cells/platelets. This leads to a prothrombotic state.
Diagnosis and examination of swollen lymph nodes ppt.pptx
The document discusses the diagnosis and evaluation of lymphadenopathy. It notes that lymphadenopathy can be localized, involving a single area, or generalized, involving two or more noncontiguous areas. For unexplained lymphadenopathy, localized cases may warrant biopsy if symptoms persist for 3-4 weeks, while generalized cases almost always indicate a systemic disease and biopsy is recommended. Risk factors for lymphoma are also outlined. Proper biopsy is important for diagnosis before treatment decisions. A case study example of a patient with cervical and supraclavicular lymphadenopathy and symptoms is then presented and worked through.
CHRONIC EOSINOPHILIC LEUKEMIA
Hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL) are conditions characterized by abnormally high levels of eosinophils in the blood. Eosinophils develop from myeloid precursor cells in the bone marrow and are normally involved in fighting parasitic infections. HES can be primary, resulting from a genetic defect, or secondary to other causes like infections, allergies, or autoimmune diseases. It is diagnosed based on eosinophil levels, organ damage symptoms, and ruling out other potential causes. Treatment focuses on reducing eosinophil levels to prevent organ damage and includes steroids, chemotherapy, or targeted therapies depending on the specific variant. CEL meets additional criteria related to the
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease caused bymutation(s) in the Mediterranean fever (MEFV, pyrinmarenostrin) gene. FMF is characterized byrecurrent fever crisis combined with serosal, synovial, or cutìaneous inflammation
Similar to Mas (20)
Expression of AFP, P-sel, and MMP-9 in Cirrhosis with Portal Vein Thrombosis
Expression of AFP, P-sel, and MMP-9 in Cirrhosis with Portal Vein Thrombosis
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
Diagnosis and examination of swollen lymph nodes ppt.pptx
Diagnosis and examination of swollen lymph nodes ppt.pptx
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
A Mild form of Familial Mediterranean Fever Associated with a Polymorphisms C...
Recently uploaded
BÀI TẬP DẠY THÊM TIẾNG ANH LỚP 7 CẢ NĂM FRIENDS PLUS SÁCH CHÂN TRỜI SÁNG TẠO ...
BÀI TẬP DẠY THÊM TIẾNG ANH LỚP 7 CẢ NĂM FRIENDS PLUS SÁCH CHÂN TRỜI SÁNG TẠO ...Nguyen Thanh Tu Collection
https://app.box.com/s/qhtvq32h4ybf9t49ku85x0n3xl4jhr15Philippine Edukasyong Pantahanan at Pangkabuhayan (EPP) Curriculum
(𝐓𝐋𝐄 𝟏𝟎𝟎) (𝐋𝐞𝐬𝐬𝐨𝐧 𝟏)-𝐏𝐫𝐞𝐥𝐢𝐦𝐬
𝐃𝐢𝐬𝐜𝐮𝐬𝐬 𝐭𝐡𝐞 𝐄𝐏𝐏 𝐂𝐮𝐫𝐫𝐢𝐜𝐮𝐥𝐮𝐦 𝐢𝐧 𝐭𝐡𝐞 𝐏𝐡𝐢𝐥𝐢𝐩𝐩𝐢𝐧𝐞𝐬:
- Understand the goals and objectives of the Edukasyong Pantahanan at Pangkabuhayan (EPP) curriculum, recognizing its importance in fostering practical life skills and values among students. Students will also be able to identify the key components and subjects covered, such as agriculture, home economics, industrial arts, and information and communication technology.
𝐄𝐱𝐩𝐥𝐚𝐢𝐧 𝐭𝐡𝐞 𝐍𝐚𝐭𝐮𝐫𝐞 𝐚𝐧𝐝 𝐒𝐜𝐨𝐩𝐞 𝐨𝐟 𝐚𝐧 𝐄𝐧𝐭𝐫𝐞𝐩𝐫𝐞𝐧𝐞𝐮𝐫:
-Define entrepreneurship, distinguishing it from general business activities by emphasizing its focus on innovation, risk-taking, and value creation. Students will describe the characteristics and traits of successful entrepreneurs, including their roles and responsibilities, and discuss the broader economic and social impacts of entrepreneurial activities on both local and global scales.
BBR 2024 Summer Sessions Interview Training
Qualitative research interview training by Professor Katrina Pritchard and Dr Helen Williams
Traditional Musical Instruments of Arunachal Pradesh and Uttar Pradesh - RAYH...
Traditional Musical Instruments of Arunachal Pradesh and Uttar Pradesh
The History of Stoke Newington Street Names
Presented at the Stoke Newington Literary Festival on 9th June 2024
www.StokeNewingtonHistory.com
Beyond Degrees - Empowering the Workforce in the Context of Skills-First.pptx
Iván Bornacelly, Policy Analyst at the OECD Centre for Skills, OECD, presents at the webinar 'Tackling job market gaps with a skills-first approach' on 12 June 2024
Main Java[All of the Base Concepts}.docx
This is part 1 of my Java Learning Journey. This Contains Custom methods, classes, constructors, packages, multithreading , try- catch block, finally block and more.
বাংলাদেশ অর্থনৈতিক সমীক্ষা (Economic Review) ২০২৪ UJS App.pdf
বাংলাদেশের অর্থনৈতিক সমীক্ষা ২০২৪ [Bangladesh Economic Review 2024 Bangla.pdf] কম্পিউটার , ট্যাব ও স্মার্ট ফোন ভার্সন সহ সম্পূর্ণ বাংলা ই-বুক বা pdf বই " সুচিপত্র ...বুকমার্ক মেনু 🔖 ও হাইপার লিংক মেনু 📝👆 যুক্ত ..
আমাদের সবার জন্য খুব খুব গুরুত্বপূর্ণ একটি বই ..বিসিএস, ব্যাংক, ইউনিভার্সিটি ভর্তি ও যে কোন প্রতিযোগিতা মূলক পরীক্ষার জন্য এর খুব ইম্পরট্যান্ট একটি বিষয় ...তাছাড়া বাংলাদেশের সাম্প্রতিক যে কোন ডাটা বা তথ্য এই বইতে পাবেন ...
তাই একজন নাগরিক হিসাবে এই তথ্য গুলো আপনার জানা প্রয়োজন ...।
বিসিএস ও ব্যাংক এর লিখিত পরীক্ষা ...+এছাড়া মাধ্যমিক ও উচ্চমাধ্যমিকের স্টুডেন্টদের জন্য অনেক কাজে আসবে ...
clinical examination of hip joint (1).pdf
described clinical examination all orthopeadic conditions .
How to deliver Powerpoint Presentations.pptx
"How to make and deliver dynamic presentations by making it more interactive to captivate your audience attention"
Solutons Maths Escape Room Spatial .pptx
Solutions of Puzzles of Mathematics Escape Room Game in Spatial.io
ISO/IEC 27001, ISO/IEC 42001, and GDPR: Best Practices for Implementation and...
Denis is a dynamic and results-driven Chief Information Officer (CIO) with a distinguished career spanning information systems analysis and technical project management. With a proven track record of spearheading the design and delivery of cutting-edge Information Management solutions, he has consistently elevated business operations, streamlined reporting functions, and maximized process efficiency.
Certified as an ISO/IEC 27001: Information Security Management Systems (ISMS) Lead Implementer, Data Protection Officer, and Cyber Risks Analyst, Denis brings a heightened focus on data security, privacy, and cyber resilience to every endeavor.
His expertise extends across a diverse spectrum of reporting, database, and web development applications, underpinned by an exceptional grasp of data storage and virtualization technologies. His proficiency in application testing, database administration, and data cleansing ensures seamless execution of complex projects.
What sets Denis apart is his comprehensive understanding of Business and Systems Analysis technologies, honed through involvement in all phases of the Software Development Lifecycle (SDLC). From meticulous requirements gathering to precise analysis, innovative design, rigorous development, thorough testing, and successful implementation, he has consistently delivered exceptional results.
Throughout his career, he has taken on multifaceted roles, from leading technical project management teams to owning solutions that drive operational excellence. His conscientious and proactive approach is unwavering, whether he is working independently or collaboratively within a team. His ability to connect with colleagues on a personal level underscores his commitment to fostering a harmonious and productive workplace environment.
Date: May 29, 2024
Tags: Information Security, ISO/IEC 27001, ISO/IEC 42001, Artificial Intelligence, GDPR
-------------------------------------------------------------------------------
Find out more about ISO training and certification services
Training: ISO/IEC 27001 Information Security Management System - EN | PECB
ISO/IEC 42001 Artificial Intelligence Management System - EN | PECB
General Data Protection Regulation (GDPR) - Training Courses - EN | PECB
Webinars: https://pecb.com/webinars
Article: https://pecb.com/article
-------------------------------------------------------------------------------
For more information about PECB:
Website: https://pecb.com/
LinkedIn: https://www.linkedin.com/company/pecb/
Facebook: https://www.facebook.com/PECBInternational/
Slideshare: http://www.slideshare.net/PECBCERTIFICATION
Pengantar Penggunaan Flutter - Dart programming language1.pptx
Pengantar Penggunaan Flutter - Dart programming language1.pptx
How to Create a More Engaging and Human Online Learning Experience
How to Create a More Engaging and Human Online Learning Experience Wahiba Chair Training & Consulting
Wahiba Chair's Talk at the 2024 Learning Ideas Conference. Your Skill Boost Masterclass: Strategies for Effective Upskilling
Your Skill Boost Masterclass: Strategies for Effective UpskillingExcellence Foundation for South Sudan
Strategies for Effective Upskilling is a presentation by Chinwendu Peace in a Your Skill Boost Masterclass organisation by the Excellence Foundation for South Sudan on 08th and 09th June 2024 from 1 PM to 3 PM on each day.Recently uploaded (20)
BÀI TẬP DẠY THÊM TIẾNG ANH LỚP 7 CẢ NĂM FRIENDS PLUS SÁCH CHÂN TRỜI SÁNG TẠO ...
BÀI TẬP DẠY THÊM TIẾNG ANH LỚP 7 CẢ NĂM FRIENDS PLUS SÁCH CHÂN TRỜI SÁNG TẠO ...
Philippine Edukasyong Pantahanan at Pangkabuhayan (EPP) Curriculum
Philippine Edukasyong Pantahanan at Pangkabuhayan (EPP) Curriculum
Traditional Musical Instruments of Arunachal Pradesh and Uttar Pradesh - RAYH...
Traditional Musical Instruments of Arunachal Pradesh and Uttar Pradesh - RAYH...
Beyond Degrees - Empowering the Workforce in the Context of Skills-First.pptx
Beyond Degrees - Empowering the Workforce in the Context of Skills-First.pptx
বাংলাদেশ অর্থনৈতিক সমীক্ষা (Economic Review) ২০২৪ UJS App.pdf
বাংলাদেশ অর্থনৈতিক সমীক্ষা (Economic Review) ২০২৪ UJS App.pdf
B. Ed Syllabus for babasaheb ambedkar education university.pdf
B. Ed Syllabus for babasaheb ambedkar education university.pdf
ISO/IEC 27001, ISO/IEC 42001, and GDPR: Best Practices for Implementation and...
ISO/IEC 27001, ISO/IEC 42001, and GDPR: Best Practices for Implementation and...
NEWSPAPERS - QUESTION 1 - REVISION POWERPOINT.pptx
NEWSPAPERS - QUESTION 1 - REVISION POWERPOINT.pptx
Pengantar Penggunaan Flutter - Dart programming language1.pptx
Pengantar Penggunaan Flutter - Dart programming language1.pptx
How to Create a More Engaging and Human Online Learning Experience
How to Create a More Engaging and Human Online Learning Experience
Your Skill Boost Masterclass: Strategies for Effective Upskilling
Your Skill Boost Masterclass: Strategies for Effective Upskilling
Mas
- 1. “ Terrible T-cells and Troubling Thrombocytopenia” Rheumatology Case Conference Case and Review of Literature 12/11/2009 Mark Gorelik Fellow, Pediatric Rheumatology
- 10. There are multiple bilateral foci of T2 and FLAIR signal hyperintensity associated withareas of restricted diffusion. They are present in bilateral frontal lobes parasagittally, with 2 on the left and 1 on the right; in the bilateral occipital lobes with 3-4 foci on the right and 1 on the left…consistent with multifocal infarctions.
- 18. Bone marrow aspirate with myeloid hyperplasia
- 19. Bone Marrow Aspirate: Histiocytes (arrows) with phagocytosed red blood cells
- 21. Evangelista, F; Blood 1999;63:1991 CDC website
- 24. Normal response to infection
- 25. Macrophage Activation Syndrome Edward M. Behrens Macrophage activation syndrome in rheumatic disease: What is the role of the antigen presenting cell? Autoimmunity reviews, Volume 7, Issue 4, Feb 2008, 305-308
- 26. Macrophage Activation Syndrome Online presentation
- 28. List of effector proteins: Src kinases, Zap70, Lat, SLP-76, PLAC-gamma, ADAP, Dynein, FMNL1, Dia1, IQGAP, Cdc42, Pyk2, Erk, HDAC6, Rab27a, AP-3, Munc 13-4, Syntaxin-11, Slp1, Slp2, Paxillin, PKC-delta, WIP, Rab3d, Rab19, Syntaxin-4, SNAP23, WASP, Syntaxin-6, Perforin
- 37. Fautrel, B, Diagnostic value of Ferritin and Glycosylated Ferritin in AOSD, J. Rheum 2001 28:2;
Editor's Notes
- Study in secondary HLH showed that lower percentage of glycosylated ferritin (~20%) in HLH confirmed patients, compared to HLH suspected but ‘unconfirmed’ had sensitivity of 86% and specifity of 71%, PPV of 91% and NPV of 63% ‘ Confirmed’ HLH included absent or decreased NK cell activity among 8 other strict criteria