We describe ten patients with sickle cell anemia who became acutely ill within a few days after a blood transfusion. Two patients died. In eight cases the posttransfusion detection of alloantibodies suggested that delayed hemolytic reactions to transfusion were involved in precipitating the acute illness. In some cases the illnesses mimicked vaso-occlusive crises, with bone marrow infarction, while in other cases transient biliary obstruction or transient renal insufficiency was documented. Profound anemia mimicked aplastic crises, but we observed a remarkable capacity of the bone marrow to restore the hemoglobin level without further transfusion. In view of the prevalence of delayed hemolytic transfusion reactions in these patients receiving frequent transfusions and whose red cell antigens differ from those of the white population, we suggest that efforts to more closely match recipient and donor red cell antigens would be clinically, technically, and financially advantageous. Moreover, criteria for transfusion in sickle cell anemia should be strictly scrutinized. Quantitation of transfused hemoglobin A has proved useful in confirming delayed hemolytic reactions in sickle cell disease.
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