Hereditary Spherocytosis in the Negro

Congenital spherocytosis is a disease characterized by a varying degree of anemia, microspherocytes in the peripheral blood smear, splenomegaly, and increased hemolysis in hypotonic saline solutions. Although probably recognized¹ and treated ² in the latter part of the 19th century, it was first accurately described as a clinical entity by Minkowski³ in 1900, and in 1907 Chauffard ⁴ first described the increased fragility of the erythrocytes in hypotonic saline. Since that time the disease has been thoroughly studied and reviewed by many workers.^5-7

The purpose of this paper is to report a genetic study in a Negro family in which there are members with evidence of congenital spherocytosis. The literature pertinent to the Negro race is reviewed, and some conclusions are drawn as to hereditary factors involved.

Report of Cases  Case 1.—A 10-year-old Negro boy (Fig. 1) was admitted to the Harriet Lane Home June 2, 1956,