Hereditary spherocytosis-sicklemia in the Negro; case report and study of a Negro family having multiple instances of hereditary spherocytosis.

نویسندگان

W W MARTIN

R H KOUGH

G C BRANCHE

چکیده

H EREDITARY SPHEROCYTOSIS in the Negro is a relatively rare occurrence. From a survey of the medical literature of the past 20 years, we have been able to collect 41 cases.1-’4 Some of these cases are subject to doubt as to whether they actually represent hereditary spherocytosis because of insufficient data or the presence of intercurrent disease.”12 It has been suggested by some authors that in the Negro this disease is more frequent in the female than in the male,8 and although this appears to be true in the cases thus far reported, there is no apparent explanation. In all other respects, hereditary spherocytosis in the Negro is indistinguishable from the disease in the Caucasian. However, only a few of the reported cases in the Negro contain an adequate family study, it usually having been limited to one or two siblings and, occasionally, one of the parents. With the increased incidence of case finding of hereditary spherocytosis in the Negro, it is inevitable that cases will be found in which this red blood cell defect will be combined with one or more of the hernoglobinopathies. A case of coincident hereditary spherocytosis and sickle cell trait in a five year old Puerto Rican child of obviously mixed parentage has been reported recently.’#{176} A similar case is referred to by Smith and Conley,15 but no details are given. The following case of hereditary spherocytosis and sickle cell trait is reported because of its intrinsic interest and because of the opportunity it afforded for a family study of hereditary spherocytosis in the Negro.

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