Hemoglobin C disease

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Blood cells

Hemoglobin C disease

Definition:

Hemoglobin C disease is a genetic blood disorder. It results in a chronic anemia in which the red blood cells are broken down prematurely.

Alternative Names:
Clinical hemoglobin C
Causes, incidence, and risk factors:

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Hemoglobin C is a hemoglobinopathy, caused by an inherited abnormality of the beta globin gene. 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 black people. A family history of hemoglobin C is a risk factor. In combination with sickle hemoglobin, it results in Hemoglobin SC disease, which is characterized by hemolysis (blood-cell destruction) and painful crises. It is usually milder than sickle cell disease.