Hemoglobin S-D Disease

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Abstract
Four unrelated Negro patients with hemoglobin S-D disease are described. The clinical course in these patients is characterized by chronic hemolytic anemia. Stress in these patients, 3 episodes of pregnancy and 1 episode of pneumonia, resulted in increased hemolysis requiring transfusions. No patient was observed to have acute thrombotic episodes resulting in painful crisis as observed in sickle cell anemia. Since these patients have sickled erythrocytes on routine blood smear and hemoglobin which migrates in the S position on paper electrophoresis at pH 8.6, they are frequently diagnosed as sickle cell anemia. Hemoglobin S and D may be easily separated by agar gel electrophoresis at pH 6.2. The diagnostic distinction between the 2 diseases is of importance because of the difference in prognosis. Concentration of hemoglobin S in these patients varied from 25 to 77% the remainder of the hemoglobin being D. Percentage of hemoglobin S could not be correlated with the clinical severity of the disease.

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