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[铁代谢在骨髓增生异常综合征中的作用]

[The role of iron metabolism in myelodysplastic syndromes].

作者信息

Finelli Carlo, Clissa Cristina, Stanzani Marta

出版信息

Recenti Prog Med. 2014 Mar;105(3):123-6. doi: 10.1701/1434.15874.

Abstract

Patients affected by myelodysplastic syndromes (MDS) with transfusion-dependent anemia are destined to develop iron overload. The main diagnostic tools for the diagnosis of transfusional iron overload are serum ferritin and transfusion history. In MDS several studies showed that iron overload is an independent negative prognostic factor. Deferasirox, an oral iron chelator, has shown efficacy and acceptable tolerability in MDS setting, and has also been shown to improve peripheral cytopenia in 10-20% of patients. Iron chelation therapy is recommended, after the transfusion of 20 red cell units, in low-risk MDS patients, and also in high-risk patients responding to treatment of the disease and/or candidates to receive allogeneic hematopoietic stem cell transplantation.

摘要

患有依赖输血的贫血的骨髓增生异常综合征(MDS)患者注定会发生铁过载。诊断输血性铁过载的主要诊断工具是血清铁蛋白和输血史。在MDS中,多项研究表明铁过载是一个独立的不良预后因素。地拉罗司是一种口服铁螯合剂,已在MDS患者中显示出疗效和可接受的耐受性,并且还显示可使10%-20%的患者外周血细胞减少得到改善。对于低危MDS患者,以及对疾病治疗有反应和/或有接受异基因造血干细胞移植可能的高危患者,在输注20个红细胞单位后,建议进行铁螯合治疗。

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