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Hemoglobin S-C Disease

By

Evan M. Braunstein

, MD, PhD, Johns Hopkins University School of Medicine

Last full review/revision Sep 2020
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Symptoms are similar to the symptoms of sickle cell disease Symptoms and Signs Sickle cell disease (a hemoglobinopathy) causes a chronic hemolytic anemia occurring almost exclusively in blacks. It is caused by homozygous inheritance of genes for hemoglobin (Hb) S. Sickle-shaped... read more Symptoms and Signs , but are usually less frequent and less severe. However, gross hematuria, retinal hemorrhages, and aseptic necrosis of the femoral head are common. Splenomegaly may be present.

Hemoglobin S-C disease is suspected in patients whose clinical features suggest sickle cell disease or whose red blood cells demonstrate sickling. Stained blood smears show target cells, spherocytes, and rarely sickle cells or oat-shaped cells. Sickling is identified in a sickling preparation, and hemoglobin electrophoresis establishes the diagnosis.

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