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铁过载患者的移植:磁共振成像有一席之地吗?:铁过载中的移植。

Transplantation in patients with iron overload: is there a place for magnetic resonance imaging? : Transplantation in iron overload.

机构信息

Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61, Palaeo Faliro, Athens, Greece.

, Athens, Greece.

出版信息

Heart Fail Rev. 2018 Mar;23(2):173-180. doi: 10.1007/s10741-018-9670-7.

Abstract

In iron overload diseases (thalassemia, sickle cell, and myelodysplastic syndrome), iron is deposited in all internal organs, leading to functional abnormalities. Hematopoietic stem cell transplantation (HSCT) is the only treatment offering a potential cure in these diseases. Our aim was to describe the experience in the field and the role of magnetic resonance imaging in the evaluation of iron overload before and after HSCT. Magnetic resonance imaging (MRI), using T2*, is the most commonly used tool to diagnose myocardial-liver iron overload and guide tailored treatment. Currently, HSCT offers complete cure in thalassemia major, after overcoming the immunologic barrier, and should be considered for all patients who have a suitable donor. The overall thalassemia-free survival of low-risk, HLA-matched sibling stem cell transplantation patients is 85-90%, with a 95% overall survival. The problems of rejection and engraftment are improving with the use of adequate immunosuppression. However, a detailed iron assessment of both heart and liver is necessary for pre- and post-transplant evaluation. In iron overload diseases, heart and liver iron evaluation is indispensable not only for the patients' survival, but also for evaluation before and after HSCT.

摘要

在铁过载疾病(地中海贫血、镰状细胞病和骨髓增生异常综合征)中,铁沉积在所有内部器官中,导致功能异常。造血干细胞移植(HSCT)是这些疾病唯一提供潜在治愈方法的治疗方法。我们的目的是描述该领域的经验以及磁共振成像在 HSCT 前后铁过载评估中的作用。磁共振成像(MRI)使用 T2*,是诊断心肌-肝脏铁过载并指导针对性治疗的最常用工具。目前,HSCT 在克服免疫障碍后为重型地中海贫血提供了完全治愈的机会,并且应该被认为是所有有合适供体的患者的首选。低危、HLA 匹配的同胞干细胞移植患者的无地中海贫血生存率为 85-90%,总生存率为 95%。随着充分免疫抑制的使用,排斥和植入问题正在得到改善。然而,对于移植前和移植后的评估,都需要对心脏和肝脏的铁进行详细评估。在铁过载疾病中,心脏和肝脏铁的评估不仅对患者的生存至关重要,而且对 HSCT 前后的评估也至关重要。

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