Hemoglobin

Sickle Cell Hemolytic Crisis

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Sickle Cell Hemolytic Crisis, Hyperhemolytic Crisis

  • See Also
  1. Sickle Cell Anemia
  2. Splenic Sequestration in Sickle Cell Anemia
  3. Transient Red Cell Aplasia
  • Pathophysiology
  1. Sickle Cell Anemia related RBC fragility predisposes to Hemolysis
  2. Increased RBC destruction may occur with Vaso-Occlusive Pain Crisis or acute infection
  • Differential Diagnosis
  1. Low Reticulocyte Count indicates an RBC production disorder
    1. Contrast with the increased Reticulocyte Count with RBC destruction in Sickle Cell Hemolytic Crisis
  2. Distinguish from more serious Anemia cause
    1. Splenic Sequestration in Sickle Cell Anemia (ages 1 to 4 years old)
    2. Transient Red Cell Aplasia
  • Findings
  1. Decreased Hemoglobin
  2. Increased Reticulocyte Count, Serum Bilirubin, and Lactate Dehydrogenase (LDH)
  • Management
  1. Treated with routine Blood Transfusion (Leukocyte depleted, irradiated) in the ED or while admitted
  2. Risk of developing antibodies that make future transfusion matching much more difficult
  3. Request extended red cell phenotyping (includes minor Blood Groups, e.g. Kell, Duffy)
  • References
  1. Lopez, Kleinmann, Chandra and Lopez (2025) Crit Dec Emerg Med 30(3): 4-12