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Blood, 1959, Vol. 14, No. 6, pp. 688-693.
© 1959 American Society of Hematology, Inc.

Hereditary Spherocytosis-Sicklemia in the Negro

Case Report and Study of a Negro Family Having Multiple Instances of Hereditary Spherocytosis

WILLIAM W. MARTIN JR. 1, ROBERT H. KOUGH 1, and GEORGE C. BRANCHE JR. 1

1 Laboratory of Clinical Investigation, the Department of Medicine, Medical College of Virginia, Richmond, Va., Hematology Section of the Medical Clinic, Hospital of the University of Pennsylvania, Philadelphia, Pa., and Richmond Community Hospital, Richmond, Va.

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.

Submitted on May 2, 1958
Accepted on November 16, 1958


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