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Recent advances in the management of Thalassemia

Abstract: The purpose of this article is to set forth the approach to diagnosing and managing the thalassemias, including thalassemia   intermedia and thalassemia major. The   article begins by briefly describing recent advances in our understanding of the pathophysiology of thalassemia. In our description of treatment strategies, we focus on how a pediatrician deal with clinical manifestations and long term complications using the most effective current treatment methods for thalassemia. The discussion of disease management focuses on the use of transfusion therapy and the newly developed oral iron chelators, deferiprone and deferasirox, especially combination of the chelator drugs. We also discusses with splenectomy and how a pediatrician manage endocrinopathies   and cardiac complications. In addition, we describe the use of hematopoietic stem cell transplantation, which has produced cure rates as high as 97%, and the use of cord blood transplantation. More recently, majo