Hemolytic Anemia II
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| show | (1)Vessel wall thickening of arterioles and capillaries (2)Swelling or detachment of endothelial cells from basement membrane (3)Material accumulation in the subendothelial space (4)Intraluminal thrombosis
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| Syndrome of thrombotic microangiopathy, thrombocytopenia, neurologic deficits, fever, and mild renal dysfunction. | show 🗑
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| show | Hemolytic Uremic Syndrome
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| Classic pentad of symptoms of Thrombotic Thrombocytopenic Purpura | show 🗑
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| show | Shiga toxin induced Hemolytic Uremic Syndrome
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| show | Diffuse endothelial damage caused by toxins (eg Shiga toxin) that in turn trigger caogulation and formation of thrombi. Increased shear stress around thrombi cause fragmentation of red cells.
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| What are the predisposing factors for renal failure in Shiga-toxin induced Hemolytic Uremic Syndrome? | show 🗑
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| Pathophysiology of renal failure caused by Shiga toxin | show 🗑
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| show | Autoantibodies inhibit ADAMTS13 causing accumulation of unusually large von Willebrane multimers (UlvWF). Shear stress of circulation exposes platelet binding sites on UlvWF, causing large thrombi and obstruction of small vessels.
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| show | ADAMTS13
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| What is the genetic syndrome associated with Thrombotic thrombocytopenic purpura? | show 🗑
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| show | Ticlopidine, Clpidogrel, Quinine, Mitomycin, Cyclosporin, and Pentostatin
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| show | (1) HIV (2) Post bone marrow transplantation (3) Cancer (4) Pregnancy (5) Autoimmune disorders (6) Abnormal components of the complement pathways (factor H)
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| show | 100%
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| show | Plasma exchange
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| (T or F) Platelets can be safely given in Thrombotic thrombocytopenic purpura and Hemolytic uremic syndrome. | show 🗑
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| What treatments for Thrombotic thrombocytopenic purpura and Hemolytic uremic syndrome can be added if plasma exchange fails? | show 🗑
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| show | Direct Coombs Test negative intravascular hemolysis
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| show | Cardiac hemolysis largely occurs with prosthetic valves especially aortic valves and those with valvular leaks.
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| show | (1) Hereditary spherocytosis (2) Hereditary Elliptocytosis (3) Hereditary pyropoikilocytosis (4) Hereditary stomatocytosis
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| What is the membrane defect in hereditary spherocytosis? | show 🗑
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| What is the membrane defect in hereditary elliptocytosis? | show 🗑
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| What is the membrane defect in hereditary pyropoikilocytosis? | show 🗑
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| What is the membrane defect in hereditary stomatocytosis? | show 🗑
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| What two RBC enzyme defects are associated with hemolytic anemia? | show 🗑
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| What is the most abundant protein in the underlying protein lattice of RBC membrane? | show 🗑
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| Pathogenesis of Hereditary Spherocytosis | show 🗑
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| What is the most common hereditary hemolytic disorder in Caucasians? | show 🗑
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| show | Hereditary Spherocytosis
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| What is the most common pattern of inheritance of hereditary spherocytosis? | show 🗑
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| show | Osmotic fragility test. Spherocytes have increased osmotic fragility.
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| Pathogenesis of Hereditary Elliptocytosis | show 🗑
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| What may cause aplastic crisis in a hereditary spherocytosis? | show 🗑
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| Treatment for symptomatic hereditary spherocytosis | show 🗑
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| What is the inheritance pattern of Hereditary Elliptocytosis? | show 🗑
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| show | Splenectomy. Most patients require no treatment.
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| Pathogenesis of Glucose-6-Phosphate Dehydrogenase Deficiency | show 🗑
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| show | Sex-linked
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| show | Type B. Almost all whites and 70% of blacks have this type of G6PD deficiency.
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| show | Ingestion of fava beans causing life-threatening hemolysis in G6PD deficiency. Found in people of Mediterranean ancestry. Enzyme activity is absent and minor oxidative stress causes life-threatening hemolysis.
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| show | Aggregates of denatured HgB
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| Function of Embden-Meyerhof glycolytic pathway | show 🗑
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| Pathogenesis of Pyruvate Kinase Deficiency | show 🗑
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| show | Autosomal recessive
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| show | Splenectomy
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| show | (1) Enzyme screening test (2) Fluorescent spot test (3) Reticulocyte count
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