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For further information, see CMDT Part 13-09: Hemolytic Anemias

Key Features

  • Generally classified according to whether the defect is intrinsic or extrinsic to the red blood cell (RBC)

  • Intrinsic defects have been described in all components of the red blood cell, including the membrane, enzyme systems, and hemoglobin; most of these disorders are hereditary

  • External factors are immune and microangiopathic hemolytic anemias and infections of red blood cells

Clinical Findings

  • Symptoms of anemia

  • Jaundice, pigment gallstones, cholecystitis in chronic cases

  • Palpable spleen

Diagnosis

  • Serum haptoglobin may be low but is neither specific nor sensitive

  • Reticulocytosis present unless second disorder (infection, folate deficiency) superimposed on hemolysis

  • Transient hemoglobinemia occurs with intravascular hemolysis

  • Hemoglobinuria occurs when capacity for reabsorption of hemoglobin by renal tubular cells exceeded

  • Positive urine hemosiderin test indicates prior intravascular hemolysis

  • Hemoglobinemia and methemalbuminemia occur with severe intravascular hemolysis

  • Indirect bilirubin elevated, total bilirubin elevated to ≤ 4 mg/dL; higher bilirubin elevations may indicate hepatic dysfunction

  • Serum lactate dehydrogenase is elevated in microangiopathic hemolysis; may be elevated in other hemolytic anemias

Treatment

  • See Treatment of specific anemias

  • Folic acid, 1 mg orally once daily

  • Transfusions possible

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