HOMOZYGOUS HEMOGLOBIN C DISEASE: REPORT OF THREE CASES

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Abstract
Three Negro females, ranging in age from 28 to 67 years, presented a fairly uniform clinical picture consisting of mild but constant hemolytic anemia, occasional episodes of jaundice which cleared spontaneously, variable degrees of hepatomegaly and marked splenomegaly. Hematologic characteristics were: numerous target cells in the peripheral blood smear (22 to 90%), increased osmotic resistance to hypotonic saline, increased fecal urobilinogen excretion, mild reticulocytosis, and bone marrow studies compatible with hemolytic anemia. Electrophoresis in each case revealed a uniform spot characteristic of hemoglobin C. Red blood cell survival studies with Cr51 in one patient revealed a 50% red cell survival time of 21 days and ferrokinetics demonstrated normal utilization of Fe59. This disease apparently does not constitute an obstetrical problem. Family studies in 2 patients are confirmatory of the present genetic theory of transmission of hemoglobin C.