Hemolytic Anemia I: General Principles - Acquired & Hereditary
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| show | Premature destruction of red cells
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| show | Spherocytes
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| show | Extravasulcar hemolysis
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| What types of RBCs are phaogcytosed by macrophages in extravascular hemolysis? | show 🗑
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| show | Bilirubin is produced in two forms: unconjugated (indirect) and conjugated (direct) forms
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| show | Liver
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| How is bilirubin excreted from the body? | show 🗑
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| show | RBC lysis that occurs in the intravascular space and release their contents into the plasma.
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| show | (1) Trauma to RBCs (2) Complement fixation of RBC (3) Exogenous toxic factors
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| show | Schistocytes
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| show | Haptoglobin.
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| show | The complex is cleared by hepatocytes and the reticuloendothelial system
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| show | Hemoglobin is filtered by the renal glomeruli and reabsorbed by the proximal tubules where it is catabolized and the heme is converted into ferritin and hemosiderin.
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| show | Presence of (1) ferritin (2) hemosiderin (3) hemoglobinuria
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| What percentage of senescent RBCs are recycled by intravascular hemolysis? | show 🗑
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| show | Ferriheme is the protein plasma transporter of any hemoglobin that is not bound to haptoglobin or excreted by the kidneys.
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| show | Coombs Test
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| show | Coombs test is used to detect immune mediated hemolytic anemia. It employs anti-human globulin which is specifically directed aganist human IgG, IgM, IgA, and complement (C3d).
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| An antiglobulin test that detects the presence of antibody or complement on the patient's RBC. Used to detect immune mediated hemolysis. | show 🗑
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| How is the Direct Coombs Test conducted? | show 🗑
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| An antiglobulin test that detects the presence of antibody to red cells in the patient's serum. Used to detect autoimmune mediated hemolysis. | show 🗑
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| How is the Indirect Coombs Test conducted? | show 🗑
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| Pathophysiology of Warm Autoimmune Hemolytic Anemia? | show 🗑
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| show | True. Most commonly associated with complications of autoimmune disorders or hematologic malignancies espeically SLE and CLL.
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| What are the findings of Warm Autoimmune Hemolytic Anemia peripheral blood smear? | show 🗑
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| At what level of serum bilirubin does jaundice? | show 🗑
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| show | Positive DAT with IgG only or both IgG and C3b.
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| First line therapy for Warm Autoimmune Hemolytic Anemia | show 🗑
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| What constitutes failure of prednisone therapy for Warm Autoimmune Hemolytic Anemia? | show 🗑
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| show | (1) Transfusions (sparingly) (2) IV IgG (3) Danazol (4) Splenectomy (5) Immunosuprressive drugs
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| Etiology of Cold Agglutinin Disease | show 🗑
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| Pathophysiology of Cold Agglutinin Disease | show 🗑
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| (T or F) Most people have cold IgM antibodies which bind their own RBCs. | show 🗑
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| show | (1) Antibody has a much higher thermal amplitude (28-31C) (2) Antibody has a much higher titier than normal cold antibody (3) Antibody is a monoclonal IgM and often the patient has an underlying lymphoreticular disorder.
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| show | (1) Episodes of hemoglobinuria and intravascular hemolysis after cold exposure (2) Acrocyanosis and Raynaud's phenomenon upon cold exposure
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| What are the findings on peripheral blood smear characteristic of Cold Agglutinin Disease? | show 🗑
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| show | Positive DAT with broad-spectrum Coomb's reagent and complement Coomb's reagent
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| show | (1) Peripheral blood smear demonstrating mild to moderate anemia, spherocytes, agglutination of RBCs (2) Positive DAT (3) Elevated cold agglutinin titer with high thermal amplitude (3) Monoclonal IgM on immunoelectropheresis
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| show | Acute Postinfectious Cold Agglutinin-Induced Hemolysis
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| Infections most commonly associated with Acute Postinfectious Cold Agglutinin-Induced Hemolysis | show 🗑
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| show | False. The cold antibodies are heterogeneous unlike cold agglutinin disease.
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| What are the laboratory findings for the diagnosis of Acute Postinfectious Cold Agglutinin-Induced Hemolysis? | show 🗑
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| Therapy for Acute Postinfectious Cold Agglutinin-Induced Hemolysis | show 🗑
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| show | IgG antibody that is biphasic hemolysin. The antibody binds to the P antigen on RBC membrane at cold temperature and activates complement at warmer temperature causing intrvascular hemolysis.
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| The most common causes of Paroxysmal Cold Hemogloinuria | show 🗑
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| show | Paroxysmal Cold Hemogloinuria
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| How is the Donath-Landsteiner Test conducted? | show 🗑
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| show | Extravascular hemolysis
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| show | Drug binds to RBC. RBC acts as the hapten and the drug as the antigen. IgG antibodies are formed aganist the antigen. The complement pathway is not activated.
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| show | DAT would be positive only for IgG.
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| show | There are IgM antibodies against a drug present in the plasma. Drug-antibody interactions create circulating immune complexes that bind to RBC via complement receptor resulting in lysis of the RBC.
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| What type of hemolysis is associated with Immune Complex Mediated drug induced hemolytic anemia? | show 🗑
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| show | Positive DAT only for complement
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| show | The drug damages RBC membrane resulting in nonspecific binding of plasma proteins.
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| Pathophysiology of Autoimmune Mechanism drug induced hemolytic anemia. | show 🗑
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| Will the Direct Coombs Test be negative after discontinuation of the offending drug in autoimmune mediated drug induced hemolytic anemia? | show 🗑
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| What explains the different clinical syndromes seen with drug-induced antibodies causing hemolytic anemia? | show 🗑
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