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Hemoglobin C Disease

By Evan M. Braunstein, MD, PhD, Assistant Professor of Medicine, Division of Hematology, Department of Medicine, Johns Hopkins School of Medicine

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Hemoglobin C disease is a hemoglobinopathy that causes symptoms of a hemolytic anemia.

The prevalence of Hb C in blacks in the US is about 2 to 3%. Heterozygotes are asymptomatic. Homozyotes have chronic hemolytic anemia and splenomegaly and symptoms consistent with anemia. Cholelithiasis is the most common complication, and splenic sequestration is possible.

Hemoglobin C disease is suspected in all patients with a family history and evidence of a hemolytic anemia, particularly in adults with splenomegaly. The anemia is usually mild but can be severe. The smear is normocytic, with frequent target cells, spherocytes, and, rarely, crystal-containing RBCs. Nucleated RBCs may be present. The RBCs do not sickle. On electrophoresis, the Hb is type C. In heterozygotes, the only laboratory abnormality is centrally targeted RBCs.

No specific treatment is recommended. Anemia usually is not severe enough to require blood transfusion.