Definition for thalassemia major

Thalassemia (American English) or thalassaemia (British English) is a dominant trait inherited disease of the red blood cells. more

Thalassemia (American English) or thalassaemia (British English) is a dominant trait inherited disease of the red blood cells. In thalassemia, the genetic defect results in reduced rate of synthesis of normal globin chains (c.f. hemoglobinopathy, which is a structural change in a globin chain leading to instability or abnormal oxygen transport). The blood cells are vulnerable to mechanical injury and die easily. Blood transfusions on a regular basis (two to three week intervals) are used by many patients to cope with the disease and maintain a healthier lifestyle than living with no treatment; bone marrow transplants can be performed if the transfusion's main side effect - build-up of iron - itself begins to be a problem. A bone marrow transplant requires careful matching to avoid rejection and further complications. less