(See also Overview of Hemolytic Anemia Overview of Hemolytic Anemia At the end of their normal life span (about 120 days), red blood cells (RBCs) are removed from the circulation. Hemolysis is defined as premature destruction and hence a shortened RBC life span... read more .)
The heterozygous S-C combination is more common than homozygous Hb C disease Hemoglobin C Disease Hemoglobin C disease is a hemoglobinopathy that causes symptoms of a hemolytic anemia. (See also Overview of Hemolytic Anemia.) The prevalence of detectable hemoglobin (Hb) C in people in the... read more . This is because of the high prevalence of hemoglobin (Hb) S trait (which is responsible for sickle cell disease Sickle Cell Disease Sickle cell disease (a hemoglobinopathy) causes a chronic hemolytic anemia occurring almost exclusively in people with African ancestry. It is caused by homozygous inheritance of genes for hemoglobin... read more ), particularly in people with African ancestry. The anemia in Hb S-C disease is milder than the anemia in sickle cell disease; some patients even have normal hemoglobin levels.
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 people with African ancestry. It is caused by homozygous inheritance of genes for hemoglobin... read more , 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.
Treatment can be similar to the treatment of sickle cell disease Treatment Sickle cell disease (a hemoglobinopathy) causes a chronic hemolytic anemia occurring almost exclusively in people with African ancestry. It is caused by homozygous inheritance of genes for hemoglobin... read more but is determined by the severity of the symptoms.