Postgraduate Research Institute of Science, Technology, Environment and Medicine Limassol, Cyprus.
Postgraduate Research Institute of Science, Technology, Environment and Medicine Limassol, Cyprus,
Front Biosci (Landmark Ed). 2018 Jan 1;23(6):1082-1098. doi: 10.2741/4634.
Iron overload toxicity is the main cause of mortality and morbidity in thalassaemia patients. The complete elimination and prevention of iron overload is the main aim of chelation therapy, which can be achieved by chelation protocols that can effectively remove excess iron load and maintain body iron at normal levels. Deferiprone and selected combinations with deferoxamine can be designed, adjusted and used effectively for removing all excess stored iron and for maintaining normal iron stores (NIS) in different categories of thalassaemia patients. High doses of deferiprone (75-100 mg/kg/day) and deferoxamine (50-60 mg/kg, 1-7 days/week) combinations can be used for achieving and maintaining NIS in heavily iron loaded transfused patients. In contrast, deferiprone (75-100 mg/kg/day) can be used effectively and sometimes intermittently for maintaining NIS in non heavily transfused patients. Deferasirox can in particular be used in patients not tolerating deferoxamine and deferiprone. The design of tailored made personalised protocols using deferiprone and selected combinations with deferoxamine should be considered as optimum chelation therapies for the complete treatment and the prevention of iron overload in thalassaemia.
铁过载毒性是地中海贫血患者死亡和发病的主要原因。螯合疗法的主要目标是完全消除和预防铁过载,这可以通过螯合方案来实现,这些方案可以有效地去除多余的铁负荷并将体内铁维持在正常水平。可以设计、调整和有效地使用地拉罗司与去铁胺的选定组合,以去除所有多余的储存铁,并维持不同类型地中海贫血患者的正常铁储存(NIS)。高剂量的地拉罗司(75-100mg/kg/天)和去铁胺(50-60mg/kg,每周 1-7 天)联合治疗可用于在大量输血的铁负荷过重的患者中实现和维持 NIS。相比之下,地拉罗司(75-100mg/kg/天)可有效且有时间断地用于维持非大量输血患者的 NIS。对于不能耐受去铁胺和地拉罗司的患者,可以使用去铁斯琼。使用地拉罗司和去铁胺的选定组合设计定制的个体化方案应被视为地中海贫血铁过载完全治疗和预防的最佳螯合疗法。