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[铁过载与骨髓增生异常综合征]

[Iron overload and myelodysplastic syndromes].

作者信息

Rose C, Cambier N, Mahieu M, Ernst O, Fenaux P

机构信息

Service d'hématologie hôpital St-Vincent groupe hospitalier Institut catholique de Lille, boulevard de Belfort, 59020 Lille, France.

出版信息

Transfus Clin Biol. 2001 Oct;8(5):422-32. doi: 10.1016/s1246-7820(01)00193-8.

Abstract

Transfusion of RBC units, the only current treatment for many myelodysplastic syndromes, and excess intestinal absorption of Fe related to dyserythopoiesis often result in iron overload. This condition is associated with high rates of morbidity and mortality. High-risk patients include those with refractory anemia, sideroblastic anemia, 5q-syndrome, patients with a good prognosis (low or lower intermediate international prognosis score), patients having received over 100 RBC units, and patients under the age of 70. Deferoxamine, while it can prevent iron overload, is a strenuous treatment requiring 8-to-12 hour-overnight subcutaneous injections. When patients comply with the regimen, it efficiently prevents mortality due to iron overload, but must be implemented early in the disorder, usually before transfusing 20 RBC concentrates. A simple way of monitoring iron overload is to measure seric ferritin levels and record the number of RBC concentrates. The chelating treatment should be modulated according to age, MDS type, international prognosis score, number of RBC units received, ferritin levels, and most of all, patient tolerance. The direct subcutaneous approach is currently being evaluated by the French Group for Myelodysplasias for its efficiency to prevent disorders, but seems to be both efficient and well complied with (a national protocol is under way). The recent findings on the proteins implied in iron recycling by macrophages after destruction of RBCs, may in the long term, enable us to manage patients with less burdensome treatments and more effective new oral chelates.

摘要

输注红细胞单位(目前治疗许多骨髓增生异常综合征的唯一方法)以及与红细胞生成异常相关的肠道铁吸收过多,常常导致铁过载。这种情况与高发病率和高死亡率相关。高危患者包括难治性贫血患者、铁粒幼细胞贫血患者、5q综合征患者、预后良好(国际预后评分低或中低)的患者、接受超过100个红细胞单位的患者以及70岁以下的患者。去铁胺虽然可以预防铁过载,但却是一种费力的治疗方法,需要进行8至12小时的夜间皮下注射。当患者遵守治疗方案时,它能有效预防因铁过载导致的死亡,但必须在疾病早期实施,通常在输注20个红细胞浓缩物之前。监测铁过载的一种简单方法是测量血清铁蛋白水平并记录红细胞浓缩物的数量。螯合治疗应根据年龄、骨髓增生异常综合征类型、国际预后评分、接受的红细胞单位数量、铁蛋白水平,最重要的是患者耐受性进行调整。法国骨髓增生异常综合征研究小组目前正在评估直接皮下给药方法预防疾病的效果,但其似乎既有效且患者依从性良好(一项全国性方案正在进行中)。最近关于红细胞破坏后巨噬细胞参与铁循环的蛋白质的研究结果,从长远来看,可能使我们能够用负担较小的治疗方法和更有效的新型口服螯合剂来治疗患者。

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