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骨髓增生异常综合征的支持性治疗和螯合疗法:我们是在拯救生命还是仅仅在降低铁含量?

Supportive care and chelation therapy in MDS: are we saving lives or just lowering iron?

机构信息

Division of Hematology, St. Paul's Hospital and the University of British Columbia, Vancouver, BC, Canada.

出版信息

Hematology Am Soc Hematol Educ Program. 2009:664-72. doi: 10.1182/asheducation-2009.1.664.

Abstract

The myelodysplastic syndromes (MDS) are characterized by cytopenias and risk of transformation to acute myeloid leukemia (AML). Although new treatments are available, a mainstay in MDS remains supportive care, which aims to minimize the impact of cytopenias and transfusion of blood products. Red blood cell (RBC) transfusions place patients at risk of iron overload (IOL). In beta-thalassemia major (BTM), IOL from chronic RBC transfusions inevitably leads to organ dysfunction and death. With iron chelation therapy (ICT), survival in BTM improved from the second decade to near normal and correlated with ICT compliance. Effects of ICT in BTM include reversal of cardiac arrhythmias, improvement in left ventricular ejection fraction, arrest of hepatic fibrosis, and reduction of glucose intolerance. It is not clear whether these specific outcomes are applicable to MDS. Although retrospective, recent studies in MDS suggest an adverse effect of transfusion dependence and IOL on survival and AML transformation, and that lowering iron minimizes this impact. These data raise important points that warrant further study. ICT is potentially toxic and cumbersome, is costly, and in MDS patients should be initiated only after weighing potential risks against benefits until further data are available to better justify its use. Since most MDS patients eventually require RBC transfusions, the public health implications both of transfusion dependence and ICT in MDS are considerable. This paper summarizes the impact of cytopenias in MDS and treatment approaches to minimize their impact, with a focus on RBC transfusions and their complications, particularly with respect to iron overload.

摘要

骨髓增生异常综合征(MDS)的特征是细胞减少症和向急性髓性白血病(AML)转化的风险。尽管有新的治疗方法,但 MDS 的主要治疗方法仍然是支持性护理,旨在最大限度地减少细胞减少症和输血的影响。红细胞(RBC)输血会使患者面临铁过载(IOL)的风险。在重型β地中海贫血(BTM)中,慢性 RBC 输血引起的 IOL 不可避免地导致器官功能障碍和死亡。随着铁螯合疗法(ICT)的应用,BTM 的生存率从第二个十年提高到接近正常水平,并与 ICT 的依从性相关。ICT 在 BTM 中的作用包括逆转心律失常、改善左心室射血分数、阻止肝纤维化和减少葡萄糖不耐受。目前尚不清楚这些特定的结果是否适用于 MDS。尽管是回顾性的,但最近 MDS 的研究表明,输血依赖和 IOL 对生存和 AML 转化有不利影响,而降低铁含量则最大限度地减少了这种影响。这些数据提出了一些重要的观点,值得进一步研究。ICT 具有潜在的毒性和繁琐性,成本高昂,并且在 MDS 患者中只有在权衡潜在风险与收益后才能启动,直到有更多的数据更好地证明其使用的合理性。由于大多数 MDS 患者最终需要输血,因此输血依赖和 ICT 在 MDS 中的公共卫生影响是相当大的。本文总结了 MDS 中细胞减少症的影响以及减轻其影响的治疗方法,重点是 RBC 输血及其并发症,特别是铁过载。

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