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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| A syndrome of thrombotic microaniopathy and severe renal dysfunction associated with E. coli O157:H7 infection. | show 🗑
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| Classic pentad of symptoms of Thrombotic Thrombocytopenic Purpura | show 🗑
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| What is the most common cause of renal failure in children? | show 🗑
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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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| show | (1) WBC > 13,000 (2) Treatment with anti-motility agents (3) Treatment with antibiotics (4) Genetic factors
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| Pathophysiology of renal failure caused by Shiga toxin | show 🗑
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| Pathophysiology of Thrombotic Thrombocytopenic Purpura | show 🗑
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| What is the metalloprotease present in plasma that cleave unusually large von Willebrane multimers (UlvWF)? | show 🗑
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| What is the genetic syndrome associated with Thrombotic thrombocytopenic purpura? | show 🗑
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| What drugs have been associated with Thrombotic thrombocytopenic purpura and Hemolytic uremic syndrome? | show 🗑
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| What illness and conditions have been associated with Thrombotic thrombocytopenic purpura and Hemolytic uremic syndrome? | show 🗑
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| What is the mortality of Thrombotic thrombocytopenic purpura and Hemolytic uremic syndrome without treatment? | show 🗑
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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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| show | Other therapies can be added - glucocorticoids, rituximab, vincristine, staph protein A columns, and splenectomy.
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| Type of hemolysis associated with cardiace hemolysis | show 🗑
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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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| show | Decreased spectrin resulting in membrane loss, spherocyte formation and decreased deformability.
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| What is the membrane defect in hereditary elliptocytosis? | show 🗑
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| show | Defective formation of spectrin tetramers
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| show | Defect in Na permeability
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| What two RBC enzyme defects are associated with hemolytic anemia? | show 🗑
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| show | Spectrin
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| show | Decreased amount of spectrin in RBC membrane causing decreased attachment of protein lattice to lipid bilayer. This results in spherocyte formation, which are poorly deformable. Decrease in the amount of membrane results in increased osmotic fragility.
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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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| What is a confirmatory test for Hereditary Spherocytosis? | show 🗑
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| Pathogenesis of Hereditary Elliptocytosis | show 🗑
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| show | Parvovirus B19 infection
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| show | Splenectomy
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| What is the inheritance pattern of Hereditary Elliptocytosis? | show 🗑
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| Treatment for symptomatic Hereditary Elliptocytosis | show 🗑
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| show | Hydrogen peroxide accumulates due to inadequate glutathione. Oxidative damage to hemoglobin by excess hydrogen peroxide causes Heinz body formation. Hydrogen peroxide also damages RBC membrane. Both intravascular and extravascular hemolysis occur.
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| What is the inheritance pattern of Glucose-6-Phosphate Dehydrogenase Deficiency? | show 🗑
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| What is the most common variant of Glucose-6-Phosphate Dehydrogenase Deficiency? | show 🗑
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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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| What are Heinz bodies? | show 🗑
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| Function of Embden-Meyerhof glycolytic pathway | show 🗑
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| show | Cannot produce adequate amounts of ATP via the Embden-Meyerhoff glycolytic pathway affecting stability and deformability of the RBC.
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| What is the inheritance pattern of Pyruvate Kinase Deficiency? | show 🗑
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| Treatment of severe hemolysis due to Pyruvate Kinase Deficiency | show 🗑
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| show | (1) Enzyme screening test (2) Fluorescent spot test (3) Reticulocyte count
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