Abstract
A hemolytic transfusion reaction occurring in a patient with sickle cell anemia provided insight into the handling of massive amounts of hemoglobin in patients who lack haptoglobin. Despite the acute intravascular release of 33 gms of hemoglobin, the peak plasma hemochromogen was only 134 mg/100 ml and only 6 per cent of the hemoglobin load was recovered in the urine. In contrast, the serum bilirubin rose sharply to a peak of 30 mg/100 ml within three hours of hemolysis.The minimal hemoglobinuria, the relatively low peak level of plasma hemoglobin, and the marked and rapid elevation of serum bilirubin suggest an increased hemoglobin clearing capacity of the chronically stimulated reticuloendothelial system in patients with chronic severe hemolytic disease.
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