Beta thalasemia: Epidemiology, diagnostic and treatment approach in Iran

Beta thalassemia is most monogenic disorders with autosomal recessive inheritance in worldwide. In this article we reviewed fifty articles about epidemiology and diagnostic and treatment procedures of beta thalassemia during 1982- 2011 and emphasis the attention to different diagnostic nad treatment procedures in Iran. Beta thalassemia is more prevalent in sea coast especially Mediterranean sea. In Iran، more cases of beta thalassemia were found in Caspian Sea and Persian Gulf regions. Also other regions including Isfahan، Fars and Bushehr have significant thalassemic patients. The IVS II-1 mutation is most prevalent variant in different regions of Iran. The diagnostic tests based on PCR play great role in identification of new mutations، heterozygote patients and pre-natal diagnosis. The molecular methods are selected depending on the mutation. Given the importance of early treatment of thalassemic patients، screening and identification of affected people is so important. At now، the common therapeutic approaches of beta thalassemia are transfusion and ferrochellator drugs. The definitive treatment of patients in case of finding HLA matched donors are bone marrow transplantation.