Acquired Hemolytic Anemias and Thalassemia
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Questions and Answers

Which of the following is a possible cause of Cold Agglutinin Disease?

  • Mycoplasma infection (correct)
  • Bacterial endocarditis
  • Autoimmune diseases
  • Viral hepatitis

    • What type of infection is associated with Cold Agglutinin Disease?

  • Fungal infections
  • Staphylococcus aureus
  • Parasitic infections
  • Mycoplasma (correct)

    • Cold Agglutinin Disease has an association with which of the following conditions?

  • Idiopathic thrombocytopenic purpura
  • Lymphoproliferative diseases (correct)
  • Celiac disease
  • Systemic lupus erythematosus

    • Cold Agglutinin Disease is categorized as which of the following?

    <p>An idiopathic condition</p> Signup and view all the answers

    Which one of these diseases is not associated with Cold Agglutinin Disease?

    <p>Celiac disease</p> Signup and view all the answers

    Which of the following infections can cause cold antibody-mediated hemolysis?

    <p>Mycoplasma pneumoniae</p> Signup and view all the answers

    At what temperature do warm antibodies optimally react with red blood cells?

    <p>37°C</p> Signup and view all the answers

    Which of the following is NOT a cause of warm antibody-mediated hemolytic anemia (AIHA)?

    <p>Mycoplasma pneumoniae</p> Signup and view all the answers

    What is the term for hemolysis induced by drugs?

    <p>Drug-related hemolysis</p> Signup and view all the answers

    Which of the following conditions is associated with allo-immune hemolytic anemia?

    <p>Hemolytic disease of the newborn</p> Signup and view all the answers

    Which type of antibody is typically involved in the pathogenesis of Paroxysmal Nocturnal Hemoglobinuria (PNH)?

    <p>IgM</p> Signup and view all the answers

    What consequence might occur when IgM leads to red cell agglutination in colder extremities?

    <p>Cyanosis and ischemia in extremities</p> Signup and view all the answers

    Which proteins are missing from the red cells in Paroxysmal Nocturnal Hemoglobinuria?

    <p>CD55 and CD59</p> Signup and view all the answers

    What laboratory sign is typically observed in a blood smear for patients with PNH?

    <p>RBC agglutination</p> Signup and view all the answers

    Which condition is commonly associated with a falsely high MCV, MCH, and MCHC in patients with PNH?

    <p>RBC agglutination</p> Signup and view all the answers

    What triggers may initiate bouts of hemolysis in PNH patients?

    <p>Exercise</p> Signup and view all the answers

    Which of the following is a less common but potential progression of PNH?

    <p>Aplastic anemia</p> Signup and view all the answers

    What is a characteristic urinary finding in patients with PNH?

    <p>Hemoglobinuria</p> Signup and view all the answers

    What is a hallmark feature of Microangiopathic Hemolytic Anemias?

    <p>Presence of schistocytes on the peripheral smear</p> Signup and view all the answers

    Which diagnostic test is used for detecting the deficiency of CD55 and CD59 in red cells?

    <p>Flow cytometry</p> Signup and view all the answers

    What is the cause of red blood cell destruction in individuals with PNH?

    <p>Complement system attacking red blood cells lacking protective proteins</p> Signup and view all the answers

    Which of the following is NOT a cause of Microangiopathic Hemolytic Anemias?

    <p>Macrocytic anemia</p> Signup and view all the answers

    Which test was historically used as the definitive diagnostic test for PNH?

    <p>Ham's test</p> Signup and view all the answers

    Which feature is usually NOT seen in the diagnosis of Non-Immune Hemolytic Anemia?

    <p>Positive DAT</p> Signup and view all the answers

    Which of the following activities has NOT been associated with mechanical damage to red cells causing hemoglobinuria?

    <p>Swimming</p> Signup and view all the answers

    What year did two track runners report dark urine after races?

    <p>1964</p> Signup and view all the answers

    Which condition can cause hemolysis due to IgM leading to red cell agglutination in the cold?

    <p>Cold-induced hemolysis</p> Signup and view all the answers

    Which of the following is a cause of hemolysis associated with chemical agents?

    <p>Arsine gas</p> Signup and view all the answers

    Which type of polycythemia is influenced by the patient's age?

    <p>Absolute</p> Signup and view all the answers

    Which substance can accumulate from dialysis fluid exposed to certain pipes, leading to hemolysis?

    <p>Copper</p> Signup and view all the answers

    Which type of lysis is classified under acquired hemolytic anemias?

    <p>Both intravascular and extravascular lysis</p> Signup and view all the answers

    What is necessary to confirm a heterozygote carrier of beta thalassemia?

    <p>Quantitation of HbA2 by HPLC</p> Signup and view all the answers

    Which routine chemistry test is typically elevated in thalassemia major and intermedia?

    <p>Indirect bilirubin</p> Signup and view all the answers

    Why are iron status, total iron binding capacity, and ferritin levels assessed in suspected thalassemia cases?

    <p>To differentiate thalassemia from iron deficiency anemia</p> Signup and view all the answers

    What is the role of globin chain testing in thalassemia diagnosis?

    <p>To determine the ratio of globin chains produced</p> Signup and view all the answers

    In immune-mediated hemolysis, how are RBCs coated for recognition by the reticuloendothelial system (RES)?

    <p>By IgG or complement</p> Signup and view all the answers

    What happens when RBC phagocytosis is incomplete due to IgG?

    <p>Spherocytes result</p> Signup and view all the answers

    What results when RBCs are coated with IgM in immune-mediated hemolysis?

    <p>Autoagglutination</p> Signup and view all the answers

    What characterizes polycythemia?

    <p>Increase in red cell mass</p> Signup and view all the answers

    What is primary polycythemia also known as?

    <p>Polycythemia vera</p> Signup and view all the answers

    Which of the following conditions is most likely to cause relative polycythemia?

    <p>Dehydration</p> Signup and view all the answers

    What is the red cell mass status in relative polycythemia?

    <p>Normal</p> Signup and view all the answers

    Which factor is NOT associated with causing secondary polycythemia?

    <p>Plasma loss</p> Signup and view all the answers

    Which age group is relative polycythemia far more common in?

    <p>Young or middle-aged men</p> Signup and view all the answers

    What is commonly found in the bone marrow of neonates with polycythemia?

    <p>Mild fibrosis in some cases</p> Signup and view all the answers

    Which blood smear characteristic is typical of polycythemia in neonates?

    <p>Packed film appearance</p> Signup and view all the answers

    Which of the following is increased in the blood film of most polycythemia cases?

    <p>Neutrophil count</p> Signup and view all the answers

    What is usually low in primary polycythemia (PV)?

    <p>Serum erythropoietin</p> Signup and view all the answers

    What feature is increased in circulating blood in polycythemia?

    <p>Erythroid progenitors</p> Signup and view all the answers

    Which element is absent in the bone marrow of neonates with polycythemia?

    <p>Stainable iron</p> Signup and view all the answers

    Which hematologic mutation is found in nearly 100% of patients with polycythemia vera?

    <p>JAK2</p> Signup and view all the answers

    Which of the following is NOT a typical clinical feature of polycythemia vera?

    <p>Weight gain</p> Signup and view all the answers

    What is the major cause of death in 10-40% of patients with polycythemia vera?

    <p>Thrombosis</p> Signup and view all the answers

    What percentage of patients with polycythemia vera typically present with splenomegaly?

    <p>75%</p> Signup and view all the answers

    Which of the following laboratory findings is typical in polycythemia vera?

    <p>Increased neutrophil alkaline phosphatase (NAP) score</p> Signup and view all the answers

    At what age is polycythemia vera most commonly diagnosed?

    <p>Over 55 years</p> Signup and view all the answers

    What symptom is characteristically triggered after a hot bath in patients with polycythemia vera?

    <p>Itching</p> Signup and view all the answers

    By what mechanism does polycythemia vera increase the risk of blood clots?

    <p>Decreased blood flow and increased blood thickness</p> Signup and view all the answers

    What is a potential cause of delayed cord clamping in infants?

    <p>Twin to twin transfusion syndrome</p> Signup and view all the answers

    Which condition is associated with secondary polycythemia due to physiological erythropoietin increase?

    <p>High altitudes</p> Signup and view all the answers

    Which is NOT a cause of polycythemia in normal healthy infants?

    <p>Heavy cigarette smoking</p> Signup and view all the answers

    Which condition can lead to a physiologically inappropriate increase in erythropoietin?

    <p>Renal cancer</p> Signup and view all the answers

    What is the cause of familial (congenital) polycythemia?

    <p>Mutations in the EPO receptor gene</p> Signup and view all the answers

    In which scenario would increased red cell mass be appropriate due to erythropoietin levels?

    <p>Pulmonary disease</p> Signup and view all the answers

    Study Notes

    Acquired Hemolytic Anemias: Immune-Mediated Hemolysis

    • Immune-mediated hemolysis occurs when RBCs react with autoantibodies with or without complement, leading to premature destruction of RBCs by the reticuloendothelial system.
    • There are two types of hemolysis: intravascular and extravascular.

    Auto-Immune Hemolytic Anemias

    • Classification of auto-immune hemolytic anemias:
      • Warm antibody-mediated
      • Cold antibody-mediated
      • Paroxysmal cold hemoglobinuria
      • Drug-related hemolysis
      • Allo-immune
      • Hemolytic transfusion reactions
      • Hemolytic disease of the newborn
      • Paroxysmal Nocturnal Hemoglobinuria

    Cold Agglutinin Disease

    • Causes of cold agglutinin disease:
      • Idiopathic
      • Mycoplasma pneumoniae
      • Infectious mononucleosis
      • Lymphoproliferative disease
    • Pathogenesis of cold agglutinin disease:
      • Pathogenic antibodies are usually IgM, which bind to RBCs in the cooler extremities.
      • Complement-coated RBCs can be lysed directly within the vessel (intravascular hemolysis) or engulfed by complement receptors on macrophages within the liver (extravascular hemolysis).

    Paroxysmal Nocturnal Hemoglobinuria

    • Pathogenesis of paroxysmal nocturnal hemoglobinuria:
      • An acquired disease in which an abnormal stem cell clone gives rise to abnormal RBCs, WBCs, and platelets all missing proteins attached to the cell surface by a GPI anchor.
      • This results in chronic intravascular hemolysis with or without obvious hemoglobinuria.
    • Diagnosis of paroxysmal nocturnal hemoglobinuria:
      • Haptoglobin levels are decreased.
      • LDH levels are increased.
      • Schistocytes are present in the peripheral smear.
      • Flow cytometry using immunofluorescent staining of RBCs with a monoclonal antibody to CD55 and CD59 reveals their deficiency.

    Non-Immune Hemolytic Anemia Classification

    • Classification of non-immune hemolytic anemia:
      • Mechanical trauma to RBCs
      • Microangiopathic hemolytic anemia
      • Infections
      • Drugs, chemicals, and venoms
      • Thrombotic thrombocytopenic purpura (TTP)
      • Hemolytic uremic syndrome
      • DIC
      • Malignant hypertension
      • Eclampsia (HELLP syndrome)
      • Metastatic cancer

    Polycythemia

    • Classification of polycythemia:
      • Absolute polycythemia
      • Relative polycythemia
    • Absolute polycythemia:
      • Primary polycythemia (polycythemia vera)
      • Secondary polycythemia
    • Relative polycythemia:
      • Red cell volume is normal, but plasma volume is reduced
      • Causes: cardiovascular problems, cigarette smoking, dehydration, plasma loss

    Primary Polycythemia Vera

    • Definition: a rare myeloproliferative disorder in which there is increased production of RBCs and sometimes also of granulocytes and platelets.
    • Clinical features:
      • Headache, dyspnea, numbness or tingling in the fingers
      • Blurred vision and night sweats
      • Itching, characteristically after a hot bath
      • Redness of the skin especially the face
      • Weight loss
      • Hypertension
      • Splenomegaly
      • Thrombosis
    • Laboratory findings:
      • Hb, Hct, and RBC count are increased
      • Neutrophil leukocytosis is seen in over half of patients
      • A raised platelet count is present in about half of the patients
      • JAK2 mutation is present in the BM and PB granulocytes in nearly 100% of patients

    Secondary Polycythemia

    • Definition: an increase in RBC mass due to some other conditions
    • Causes:
      • Placental red cell transfusion
      • Placental insufficiency with increased fetal erythropoiesis secondary to intra-uterine hypoxia
      • High altitudes
      • Pulmonary disease and alveolar hypoventilation
      • Cardiovascular disease
      • Increased affinity hemoglobin
      • Heavy cigarette smoking
      • Renal cancer
      • Tumors such as uterine, hepatocellular carcinoma

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    Description

    This quiz covers acquired hemolytic anemias, immune-mediated hemolysis, and thalassemia. Topics include intravascular and extravascular lysis, quantitation of HbA2, and routine chemistry tests for thalassemia.

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