Kontoghiorghes George J
Postgraduate Research Institute Science, Technology, Environment and Medicine, Limassol, Cyprus.
Hemoglobin. 2008;32(1-2):1-15. doi: 10.1080/03630260701726533.
New developments in the area of iron and other metal metabolism and toxicity and the effects and uses of chelators have been presented at the 16th International Conference on Chelation (ICOC), Limassol, Cyprus in October 2006. Marketing practices by pharmaceutical companies, contradictory policies by regulatory authorities and ineffective policies by health authorities deprive thousands of thalassemia and other transfused patients of life saving iron chelating drugs and of efficacious chelation treatments. Thousands of patients were using deferasirox (DFRA) worldwide a few months after the European Union (EU) authorities, and about 1 year after the Food and Drugs Administration (FDA), proceeded to its accelerated approval with no sufficient evidence that the drug was efficacious, especially for clearing excess cardiac iron, and also safe. Cases of fatal, acute, irreversible renal and liver failure, fatal agranulocytosis and other toxicities have recently been reported with DFRA. The FDA has not yet approved deferiprone (L1) depriving thousands of patients of potentially life saving treatment. The high cost of DFRA at 60 euros/g, L1 at 5.5 euros/g and deferoxamine (DFO) at 8.3 euros/g, diminishes the prospects of universal chelation therapy, especially for patients in developing countries. The safety and efficacy record of L1, DFO, and their combination in particular, appear to provide universal solutions in the treatment of transfusional iron overload, and also in reducing mortality because of their ability to clear rapidly and effectively excess cardiac iron.
2006年10月在塞浦路斯利马索尔举行的第16届国际螯合作用会议(ICOC)上,展示了铁及其他金属代谢与毒性领域以及螯合剂的作用和用途方面的新进展。制药公司的营销行为、监管当局相互矛盾的政策以及卫生当局无效的政策,使成千上万的地中海贫血患者和其他输血患者无法获得挽救生命的铁螯合剂药物以及有效的螯合治疗。在欧盟当局批准后几个月,以及在美国食品药品监督管理局(FDA)批准后约一年,全球就有数千名患者开始使用地拉罗司(DFRA),但当时并没有足够的证据证明该药物有效,尤其是在清除心脏多余铁方面,而且也不安全。最近有报道称,使用DFRA出现了致命、急性、不可逆的肾和肝功能衰竭、致命性粒细胞缺乏症及其他毒性反应。FDA尚未批准去铁酮(L1),这使数千名患者失去了可能挽救生命的治疗机会。DFRA每克60欧元、L1每克5.5欧元、去铁胺(DFO)每克8.3欧元,高昂的成本降低了普遍螯合治疗的前景,尤其对于发展中国家的患者。特别是L1、DFO及其联合使用的安全性和有效性记录,似乎为输血性铁过载的治疗提供了通用解决方案,并且由于它们能够快速有效地清除心脏多余的铁,也降低了死亡率。