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