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Hemoglobin S-C Disease

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by Alan E. Lichtin, MD

Hemoglobin S-C disease is a hemoglobinopathy (see Hemoglobinopathies) that causes symptoms similar to those of sickle cell disease, but milder.

Because 10% of blacks carry the Hb S trait, the heterozygous S-C combination is more common than homozygous Hb C disease. The anemia in Hb S-C disease is milder than the anemia in sickle cell disease; some patients even have normal Hb levels. Most symptoms are those of sickle cell disease, but symptoms are usually less frequent and less severe. However, gross hematuria, retinal hemorrhages, and aseptic necrosis of the femoral head are common. Hb S-C disease is suspected in patients whose clinical features suggest sickle cell disease or whose RBCs demonstrate sickling. Stained blood smears show target cells and a rare sickle cell. Sickling is identified in a sickling preparation, and Hb electrophoresis establishes the diagnosis. Treatment can be similar to that of sickle cell disease but is determined by severity of symptoms.

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