Useful For

Clinical Significance Sickle cell anemia is an inherited disease characterized by the presence of hemoglobin S, which occurs in either the homozygous (S/S-sickle cell anemia), or heterozygous (A/S-sickle cell trait) form. In a normal adult, at least 95 percent of the hemoglobin present is hemoglobin A. Hemoglobin S, inherited in the homozygous (S/S) form, is often fatal before adolescence. However, with early detection and treatment, survival
into the adult years is possible. The most obvious clinical symptoms are severe hemolytic anemia with concurrent effects on various organ systems (i.e., spleen, kidneys, lungs, central nervous system, bones). If inherited in the heterozygous (A/S) form it is usually asymptomatic except under certain circumstances of reduced oxygen tension.