Studies on Abnormal Hemoglobins : IX. Pure (Homozygous) Hemoglobin C Disease

Four Negro patients with pure (homozygous) hemoglobin C disease are described, who exhibited the characteristic features of, (1) a hemolytic process with or without anemia, (2) splenomegaly, and, (3) numerous target cells in the film. The method of choice to establish the diagnosis objectively is paper electrophoresis of the stroma-free hemolysate, prepared from the patient’s erythrocytes. No alkali resistant (F) hemoglobin was found in this syndrome (homozygous for hemoglobin C) in contradistinction to sickle cell anemia (homozygous for hemoglobin S) where F hemoglobin is usually present. Splenectomy did not ameliorate the hemolytic process.