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Blood cells
Blood cells

Clinical hemoglobin C
Definition:
Clinical hemoglobin C is a genetic blood disorder that results in a chronicanemia in which the red blood cells are broken down prematurely.

Alternative Names:
Hemoglobin C disease

Causes, incidence, and risk factors:

Hemoglobin C is a hemoglobinopathy, caused by an inherited abnormality of the beta-hemoglobin 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 the black race. 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 and painful crises. It is usually milder than sickle cell disease.
Symptoms:
  • Usually none
  • Jaundice (yellowish color to the skin) is episodic
Signs and tests:
Physical examination reveals an enlarged spleen.
This disease may also alter the results of a hemoglobin; serum test.
Treatment:
Usually no treatment is required. Folic acid may help with the production of normal red blood cells, and supplementation may improve the symptoms of the anemia.
Expectations (prognosis):
People with clinical hemoglobin C can expect to lead a normal life.
Complications:

A complication is gallbladder disease.
Calling your health care provider:
Call your health care provider if symptoms occur, or if new symptoms develop (jaundice, abdominal pain, weakness, fatigue, or shortness of breath).
Prevention:
Genetic counseling is helpful for determining the risk for offspring in high-risk couples.
Review Date: 10/18/2001
Reviewed By: Rebecca Elstrom, M.D., Division of Hematology-Oncology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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