Clinical hemoglobin C

Alternative names:

hemoglobin C disease

Definition:

A genetic blood disorder that results in a chronic anemia in which the red blood cells are broken down prematurely.

Causes, incidence, and risk factors:

Hemoglobin C is a hemoglobinopathy that includes the production of an abnormal hemoglobin. This disorder is inherited as an autosomal recessive trait. Hemoglobin C produces a mild type of anemia that occurs because the red blood cells containing abnormal hemoglobin are broken down prematurely. Normal hemoglobin, known as hemoglobin A, is also present. The spleen may be enlarged, and there may be jaundice, a yellow color of the skin. Gallstones may develop and require treatment in adulthood. The disease occurs predominantly in the black race. A family history of hemoglobin C is a risk factor. In American blacks, the incidence is approximately 2% and is higher in blacks from Ghana.