Hemolytic Anemia Study Notes
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Questions and Answers

What does the osmotic fragility test specifically evaluate in hereditary spherocytosis?

  • The rate of hemoglobin synthesis
  • The structural integrity of hemoglobin
  • The resistance of red blood cells to hemolysis by osmotic pressure (correct)
  • The volume of red blood cells

    • Which defect is primarily responsible for hereditary elliptocytosis?

  • Decreased association of spectrin dimers to form tetramers (correct)
  • Altered lipid membrane composition
  • Complete absence of red blood cell enzymes
  • Increased hemoglobin synthesis

    • Which enzyme deficiency is most commonly linked to glycolytic pathway disorders?

  • Succinate dehydrogenase deficiency
  • Pyruvate kinase deficiency (correct)
  • Lactate dehydrogenase deficiency
  • Fructose-1,6-bisphosphatase deficiency

    • What is the critical role of glucose-6-phosphate dehydrogenase (G6PD) in erythrocytes?

    <p>To maintain glutathione levels to combat oxidative stress (C)</p> Signup and view all the answers

    What are Heinz bodies associated with?

    <p>Abnormal hemoglobin precipitation in red blood cells (C)</p> Signup and view all the answers

    During an acute hemolytic episode in G6PD deficiency, what is the recommended management approach?

    <p>Administer blood transfusions and remove the oxidizing agent (A)</p> Signup and view all the answers

    What primarily causes autoimmune hemolytic anemia (AIHA)?

    <p>Immune system attacks intrinsic red blood cell components (C)</p> Signup and view all the answers

    How do warm and cold autoimmune hemolytic anemias differ in terms of antibody involvement?

    <p>Warm AIHA reacts at body temperature, cold AIHA at lower temperatures (A)</p> Signup and view all the answers

    What is the purpose of the direct antiglobulin test (DAT)?

    <p>To detect in vivo coated erythrocytes with antibodies (A)</p> Signup and view all the answers

    Which treatment is generally effective for warm autoimmune hemolytic anemia?

    <p>Corticosteroids and potential splenectomy (B)</p> Signup and view all the answers

    What characterizes paroxysmal nocturnal hemoglobinuria (PNH)?

    <p>A consequence of a PIG-A gene somatic mutation (D)</p> Signup and view all the answers

    What is the primary treatment modality for paroxysmal nocturnal hemoglobinuria?

    <p>Eculizumab, a monoclonal antibody therapy (C)</p> Signup and view all the answers

    What leads to hemolysis in fragmentation syndromes?

    <p>Mechanical damage from turbulent blood flow (D)</p> Signup and view all the answers

    What condition is recognized for causing spur cell hemolytic anemia?

    <p>Chronic liver disease or cirrhosis (D)</p> Signup and view all the answers

    What effect do cold agglutinins have in automated blood cell counts?

    <p>They cause erythrocytes to aggregate and misrepresent counts (B)</p> Signup and view all the answers

    What is the underlying cause of red blood cell destruction in hereditary spherocytosis?

    <p>An abnormally shaped cell due to a deficiency in spectrin, resulting in increased fragility (C)</p> Signup and view all the answers

    Which of the following is NOT a common clinical manifestation of hemolytic anemia?

    <p>Hypoglycemia due to increased glucose consumption by red blood cells (B)</p> Signup and view all the answers

    Which statement best describes the primary difference between extrinsic and intrinsic hemolytic anemias?

    <p>Extrinsic anemias are caused by external factors affecting red blood cells, while intrinsic anemias arise from defects within the red blood cell itself (B)</p> Signup and view all the answers

    What is the role of the spleen in the pathogenesis of hemolytic anemias?

    <p>Filtering and removing damaged or abnormal red blood cells from circulation (D)</p> Signup and view all the answers

    Which of the following laboratory tests would be most useful in diagnosing hemolytic anemia?

    <p>Complete blood count (CBC), particularly a low red blood cell count and elevated reticulocyte count (C)</p> Signup and view all the answers

    What is the most likely explanation for the development of jaundice in hemolytic anemia?

    <p>Increased breakdown of red blood cells leads to elevated bilirubin levels, which cannot be processed by the liver (A)</p> Signup and view all the answers

    Which statement accurately describes the rate of red blood cell production in the bone marrow in response to hemolytic anemia?

    <p>The bone marrow compensates by significantly increasing red blood cell production, though it may be insufficient (B)</p> Signup and view all the answers

    Which of the following statements is NOT true about erythroid hyperplasia?

    <p>It always leads to a complete recovery from anemia (A)</p> Signup and view all the answers

    Flashcards

    Hemolytic Anemia

    Anemia caused by increased red blood cell destruction.

    Normal Red Cell Production Rate

    2-3 times the normal rate after full marrow expansion.

    Erythroid Hyperplasia

    Excessive growth of immature red blood cells.

    Intrinsic Hemolytic Anemia

    Anemia caused by intrinsic red cell defects, often inherited.

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    Extrinsic Hemolytic Anemia

    Anemia caused by extracorpuscular factors or acquired conditions.

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    Defect in Hereditary Spherocytosis

    Deficiency of spectrin, ankyrin, or band 3 protein.

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    Shape of RBCs in Hereditary Spherocytosis

    Spherical shape of red blood cells.

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    Difference between Hereditary and Acquired Anemias

    Hereditary anemias are caused by intrinsic defects; acquired are extracorpuscular.

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    Osmotic fragility test

    Measures RBC resistance to hemolysis by osmotic pressure.

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    Hereditary elliptocytosis defect

    Decreased association of spectrin dimers to form tetramers.

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    G6PD deficiency

    Most common enzyme deficiency in glycolytic pathway.

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    Function of G6PD

    Maintains levels of glutathione (GSH) for reducing oxidants.

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    Heinz bodies

    Abnormal hemoglobin precipitates in red blood cells.

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    Treatment for G6PD deficiency

    Blood transfusion and removal of the oxidizing agent during hemolytic episodes.

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    Autoimmune hemolytic anemia (AIHA) cause

    Antibody production by the body against its own red blood cells.

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    Warm vs cold AIHA

    Warm AIHA responds best at 37°C; cold AIHA at 4°C.

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    Direct antiglobulin test (DAT)

    Detects erythrocytes coated with antibody in vivo.

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    Treatment for warm AIHA

    Corticosteroids, blood transfusion, or splenectomy.

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    Paroxysmal nocturnal hemoglobinuria (PNH)

    Acquired hemolytic anemia due to a somatic mutation in PIG-A gene.

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    Primary treatment for PNH

    Eculizumab, a monoclonal antibody.

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    Hemolysis in fragmentation syndromes

    Physical damage to red cells on abnormal surfaces.

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    Cause of spur cell hemolytic anemia

    Severe impaired liver function or cirrhosis.

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    Cold agglutinins effect on blood counters

    Cause RBCs to appear macrocytic.

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    Study Notes

    Hemolytic Anemia Study Notes

    • Hemolytic Anemia Definition: Anemia caused by increased red blood cell destruction.

    • Normal Red Cell Production: Adult bone marrow produces red blood cells at a rate of 5-7 times the normal rate.

    • Erythroid Hyperplasia: Increased production of immature red blood cells.

    • Intrinsic (Hereditary) vs. Extrinsic (Acquired) Hemolytic Anemias: Hereditary anemias stem from intrinsic red blood cell defects; acquired anemias result from extra-corpuscular factors.

    Classification of Hemolytic Anemias

    • Hereditary Examples: Hereditary spherocytosis, hereditary elliptocytosis, sickle cell anemia, thalassemia.

    • Acquired Examples: Autoimmune hemolytic anemia, drug-induced hemolytic anemia, paroxysmal nocturnal hemoglobinuria (PNH), microangiopathic hemolytic anemia.

    Hereditary Hemolytic Anemias

    • Hereditary Spherocytosis: Defect in spectrin, ankyrin, or band 3 protein; red blood cells have a spherical shape. Osmotic fragility test detects increased fragility to osmotic pressure.

    • Hereditary Elliptocytosis: Defect in spectrin dimer association, causing elliptical red blood cell shape.

    Enzymatic Deficiencies

    • Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency: Common enzyme defect in the glycolytic pathway; crucial for maintaining glutathione levels to combat cellular oxidants.

    • Heinz Bodies: Abnormal hemoglobin precipitates in red blood cells.

    • Treatment for G6PD deficiency: Blood transfusion and removal of the oxidizing agent during acute hemolytic episodes.

    • Pyruvate Kinase Deficiency: Another glycolytic pathway enzyme deficiency causing hemolytic anemia.

    Immune Hemolytic Anemias

    • Autoimmune Hemolytic Anemia (AIHA): Body produces antibodies against its own red blood cells.

    • Warm vs. Cold AIHA: Warm AIHA involves antibodies reacting optimally at 37°C; cold AIHA involves antibodies reacting best at 4°C.

    • Direct Antiglobulin Test (DAT): Used to detect red blood cells coated with antibodies.

    • Treatment for Warm AIHA: Corticosteroids, blood transfusion, splenectomy.

    Acquired Hemolytic Anemias

    • Paroxysmal Nocturnal Hemoglobinuria (PNH): Acquired anemia caused by a somatic PIG-A gene mutation.

    • PNH Treatment: Eculizumab (a monoclonal antibody).

    • Red Cell Fragmentation Syndromes: Hemolysis due to physical damage on abnormal surfaces.

    • Spur Cell Anemia: Hemolytic anemia linked to severe liver dysfunction.

    Miscellaneous

    • Cold Agglutinins: Affect automated blood cell counters by making red cells appear larger.

    • Hemolytic Disease of the Newborn (HDN): Maternal antibodies attacking fetal red blood cells.

    • Drug-Induced Immune Hemolytic Anemia (Hapten Model): Drug binds to erythrocyte membrane proteins creating an immunogenic complex.

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    Description

    Explore the key concepts related to hemolytic anemia, including definitions, classifications, and examples of both hereditary and acquired forms. This quiz covers important aspects like erythroid hyperplasia and the differences between intrinsic and extrinsic hemolytic anemias.

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