Beta-thalassemia intermedia, review of literatures

Beta-thalassemia intermedia (TI) is a genetic variant of beta-thalassemias with clinical presentations vary from asymptomatic like beta-thalassemia minor to severe hemolytic and transfusion dependent phenotype like betathalassemia major. Different genetic defects are involved in this disorder and based on severity of disease clinical complications like skeletal deformities and growth retardation, splenomegaly, extramedullaryhematopoiesis, heart failure and endocrine disorders may be presented in untreated patients. Precise diagnosis and management are essential in these patients for prevention of later clinical complications. There are some treatment strategies like transfusion and iron chelation therapy, splenectomy, modulation of gamma-globulin chain production; such as hydroxyurea therapy andstem cell transplantation for management of these patients.Theaim of this literature was to review the genetic defects, major clinical complications and treatments strategies in TI