Sickle Cell Hemolytic Crisis

Aka: Sickle Cell Hemolytic Crisis, Hyperhemolytic Crisis

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II. 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

III. 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. Delayed Hemolytic Transfusion Reaction (DHTR)
    1. Hemolysis may occur with transfusion due to patient antibodies to transfused or native blood
    2. Results in Hemoglobin decrease following recent Blood Transfusion
    3. Treated with transfusion AND Immunosuppression
  3. Distinguish from more serious Anemia cause
    1. Splenic Sequestration in Sickle Cell Anemia (ages 1 to 4 years old)
    2. Transient Red Cell Aplasia

IV. Findings

  1. Decreased Hemoglobin
  2. Increased Reticulocyte Count, Serum Bilirubin, and Lactate Dehydrogenase (LDH)

V. 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)

VI. References

  1. Lopez, Kleinmann, Chandra and Lopez (2025) Crit Dec Emerg Med 30(3): 4-12

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Related Topics in Hemoglobinopathies

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