The clinic is a specialized service designed to assist the primary care provider with periodic assessments and ancillary services. The thalassemias, the most common genetic disorder in the world, are inherited disorders of hemoglobin synthesis characterized by the absence or reduced amount of one or more of the globin chains of hemoglobin. Trait individuals may have a mild anemia. Infants born with thalassemia major usually present with severe anemia during the first two years of life during which time fetal hemoglobin production declines and adult hemoglobin fails to rise normally. Accumulation of iron from transfusion however must be controlled by chelation therapy.
Splenectomy is often necessary.
Children's Hospital Oakland CA Joint Center for Sickle Cell and Thalassemic Disorders. The Sickle Information Center at Emory University. Sickle-cell anemia, a molecular disease. 2 Huntsman RG, Sickle-Cell Anemia and Thalassemia, St. John's, Newfoundland, Canada. The Canadian Sickle Cell Society.