Thalassemia Minor and Major: Current Management

Abstract

Thalassemia is a common genetic disorder. It has been estimated that in India nearly 5 crore people are thalassemia carriers. They are asymptomatic and are detected on blood tests. These people are at same risk of developing iron deficiency anemia as general population and need iron therapy in the presence of iron deficiency anemia. Nearly 12,000 children with thalassemia major (Homozygous state) are born every year. These children often present with significant anemia along with hepatosplenomegaly during infancy and require early diagnosis and institution of therapy with repeated blood transfusions and chelation therapy. Adequate dose of chelation therapy is essential to maintain serum ferritin around 1000 ng/ml. With present protocol of management, thalassemic children have near normal life. Bone marrow transplantation offers cure for these children; results of bone marrow transplantation are best when performed below 7 y of age.