Hemoglobin C, S-C, and E Diseases
Hemoglobin C, S-C, and E diseases are inherited conditions characterized by abnormally shaped red blood cells and chronic anemia that is caused by excessive destruction of red blood cells.
Hemoglobin C disease occurs mostly in blacks, affecting 2 to 3% of blacks in the United States. A person must inherit two copies of the abnormal gene that causes the disease in order to develop it. In general, symptoms are few. Anemia varies in severity. People who have this disease, particularly children, may have episodes of abdominal and joint pain, an enlarged spleen, and mild jaundice, but they do not have severe crises, as occur in sickle cell disease.
Hemoglobin S-C disease occurs in people who have one copy of the gene for sickle cell disease and one copy of the gene for hemoglobin C disease. Hemoglobin S-C disease is much more common than hemoglobin C disease, and its symptoms are similar to those of sickle cell disease but much milder.
Hemoglobin E disease affects primarily blacks and people from Southeast Asia. This disease produces anemia but none of the other symptoms that occur in sickle cell disease and hemoglobin C disease.
|
