sickle cell disease

Sickle cell disease refers to an autosomal dominant inherited hemolytic disease in which red blood cells contain hemoglobin S (HbS). There are three types of this disease: the homozygote of HbS is called sickle cell anemia; the heterozygote is called sickle cell trait; and the double heterozygote formed by the combination of HbS with thalassemia or other abnormal Hb genes is called mixed sickle cell syndrome. This disease is more common in Black populations in Africa and the Americas, and is not rare among Greeks, Turks, Middle Easterners, and Indians. It is extremely rare in China. In 1975, three adult patients were found in a family in the Foshan area of Guangdong Province, two of whom had sickle cell trait and one had sickle cell/β-thalassemia; all were of Chinese-African mixed ancestry.