SICKLE CELL-HEMOGLOBIN D DISEASE

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Abstract
A Caucasian male from North Carolina was proved to have sickle cell-hemoglobin D disease. His clinical course was characterized by anemia, recurrent jaundice, pain in the extremities and back, and repeated episodes of pneumonitis. Sickling of his erythrocytes could be produced and the electrophoretic pattern of his hemoglobin on filter paper at pH 8.6 could not be distinguished from that of sickle cell anemia. On agar plates at pH 6.2, however, there was clear separation of the S and D components, and a smaller component with the mobility of fetal hemoglobin was also demonstrated. The mother of the patient and 5 of her relatives were found to have hemoglobin D trait. The patient''s father and 6 of his relatives had the sickle cell trait. In neither lineage was there evidence of Negro ancestry. The genetic data which were obtained support the belief that hemoglobin D is an allele of the A, C and S hemoglobins.