Hereditary Spherocytosis-Sicklemia in the Negro : Case Report and Study of a Negro Family Having Multiple Instances of Hereditary Spherocytosis

1. A case of coexistent hereditary spherocytosis and sicklemia in a Negro female is reported. Hematologic and clinical amelioration followed splenectomy.

2. Study of three generations of the family of the propositus revealed one additional case of hereditary spherocytosis-sicklemia and 10 cases of hereditary spherocytosis in the Negro.

3. Further evidence is adduced that the combination of this heterozygous hemoglobinopathy with the red blood cell defect of hereditary spherocytosis produces an illness which is not grossly different from hereditary spherocytosis associated with normal hemoglobin.