Hemolytic Anemia II
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| Microscopic findings characteristic of thrombotic microangiopathy | show 🗑
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| show | Thrombotic Thrombocytopenic Purpura
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| show | Hemolytic Uremic Syndrome
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| show | (1) Microangiopathic hemolysis (2) Thrombocytopenia (Platelets <80,000 with normal PT and PTT) (3) Neurologic deficits (4) Fever (5) Renal Dysfunction
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| What is the most common cause of renal failure in children? | show 🗑
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| Pathophysiology of hemolytic uremic syndrome | show 🗑
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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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| 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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| show | Schulman-Upshaw Syndrome
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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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| 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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| show | False. Platelet transfusion is contraindicated because they may exacerbate the underlying condition and should be only given for life threatening bleeding.
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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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| What is the most common cause of cardiac hemolysis? | show 🗑
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| What inherited membrane abnormalities are associated with hemolysis? | show 🗑
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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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| show | (1) G6PD deficiency (2) Pyruvate kinase Deficiency
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| What is the most abundant protein in the underlying protein lattice of RBC membrane? | show 🗑
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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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| show | Hereditary Spherocytosis
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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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| show | Parvovirus B19 infection
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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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| Favism | show 🗑
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| What are Heinz bodies? | show 🗑
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| show | Provides energy as ATP for (1) Na/K pump affecting osmotic/volume balance (2) Phosphorylation of spectrin which affects membrane deformability
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| Pathogenesis of Pyruvate Kinase Deficiency | show 🗑
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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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