Merkel Drorit G, Nagler Arnon
Division of Hematology, Chaim Sheba Medical Center, Tel Aviv University, Tel-Hashomer, Israel.
Expert Rev Anticancer Ther. 2014 Jul;14(7):817-29. doi: 10.1586/14737140.2014.896208. Epub 2014 Mar 18.
Transfusion dependent low risk myelodysplastic syndromes (MDS) patients, eventually develop iron overload. Iron toxicity, via oxidative stress, can damage cellular components and impact organ function. In thalassemia major patients, iron chelation therapy lowered iron levels with recovery of cardiac and liver functions and significant improvement in survival. Several noncontrolled studies show inferior survival in MDS patients with iron overload, including an increase in transplant-related mortality and infection risk while iron chelation appears to improve survival in both lower risk MDS patients and in stem cell transplant settings. Collated data are presented on the pathophysiological impact of iron overload; measuring techniques and chelating agents' therapy positive impact on hematological status and overall survival are discussed. Although suggested by retrospective analyses, the lack of clear prospective data of the beneficial effects of iron chelation on morbidity and survival, the role of iron chelation therapy in MDS patients remains controversial.
依赖输血的低风险骨髓增生异常综合征(MDS)患者最终会出现铁过载。铁毒性通过氧化应激可损害细胞成分并影响器官功能。在重型地中海贫血患者中,铁螯合疗法降低了铁水平,心脏和肝脏功能得以恢复,生存率显著提高。多项非对照研究表明,铁过载的MDS患者生存率较低,包括移植相关死亡率和感染风险增加,而铁螯合似乎可改善低风险MDS患者以及干细胞移植患者的生存率。本文汇总了关于铁过载病理生理影响的数据;讨论了测量技术以及螯合剂治疗对血液学状态和总生存率的积极影响。尽管回顾性分析提出了相关建议,但由于缺乏关于铁螯合对发病率和生存率有益影响的明确前瞻性数据,铁螯合疗法在MDS患者中的作用仍存在争议。