Chakrabarty P, Rudra S, Hossain M A, Bhuiyan M R, Khaleque M A, Haque M M
Mymensingh Medical College, Mymensingh, Bangladesh.
Mymensingh Med J. 2011 Jul;20(3):513-9.
New developments in the epidemiology, treatment and prognosis of thalassemia have dramatically altered the approach to the care of affected patients. In the last 30 years, conventional treatment of β-thalassemia major, based primarily on regular blood transfusions and iron chelation therapy with desferrioxamine (DFO) has markedly improved the prognosis of the disease. Adequate administration of parenteral DFO reduces or prevents iron accumulation. The unavailability of DFO (dysferol) for most patients with thalassemia major and the failure of prescribed therapy to prevent complications in other patients have led to a search for alternative iron chelators; one of them, deferiprone (DFP) has been commercially available. Patients may ultimately benefit from having a choice between several chelators, including orally active drugs. Combination therapy and organ-targeted chelation, may soon have a considerable impact on the therapeutic outcome and quality of life of patients with thalassemia.
地中海贫血在流行病学、治疗和预后方面的新进展显著改变了对受影响患者的护理方式。在过去30年中，重度β地中海贫血的传统治疗主要基于定期输血和使用去铁胺（DFO）进行铁螯合治疗，这已显著改善了该疾病的预后。充分给予肠外DFO可减少或预防铁蓄积。大多数重度地中海贫血患者无法获得DFO（去铁酮），且规定的治疗未能预防其他患者的并发症，这促使人们寻找替代的铁螯合剂；其中一种，去铁酮（DFP）已上市。患者最终可能会从几种螯合剂（包括口服活性药物）之间的选择中受益。联合治疗和器官靶向螯合可能很快会对地中海贫血患者的治疗效果和生活质量产生重大影响。
