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对于非输血依赖型地中海贫血患者,何时考虑输血治疗。

When to consider transfusion therapy for patients with non-transfusion-dependent thalassaemia.

作者信息

Taher A T, Radwan A, Viprakasit V

机构信息

American University of Beirut, Beirut, Lebanon.

出版信息

Vox Sang. 2015 Jan;108(1):1-10. doi: 10.1111/vox.12201. Epub 2014 Oct 7.

Abstract

Non-transfusion-dependent thalassaemia (NTDT) refers to all thalassaemia disease phenotypes that do not require regular blood transfusions for survival. Thalassaemia disorders were traditionally concentrated along the tropical belt stretching from sub-Saharan Africa through the Mediterranean region and the Middle East to South and South-East Asia, but global migration has led to increased incidence in North America and Northern Europe. Transfusionists may be familiar with β-thalassaemia major because of the lifelong transfusions needed by these patients. Although patients with NTDT do not require regular transfusions for survival, they may require transfusions in some instances such as pregnancy, infection or growth failure. The complications associated with NTDT can be severe if not properly managed, and many are directly related to chronic anaemia. Awareness of NTDT is important, and this review will outline the factors that should be taken into consideration when deciding whether to initiate and properly plan for transfusion therapy in these patients in terms of transfusion interval and duration of treatment.

摘要

非输血依赖型地中海贫血(NTDT)指的是所有无需定期输血即可存活的地中海贫血疾病表型。传统上,地中海贫血症集中在从撒哈拉以南非洲延伸至地中海地区、中东以及南亚和东南亚的热带地带,但全球人口迁移导致北美和北欧的发病率上升。由于这些患者需要终身输血,输血科医生可能对重型β地中海贫血较为熟悉。虽然NTDT患者无需定期输血来维持生命,但在某些情况下,如怀孕、感染或生长发育迟缓时,他们可能需要输血。如果管理不当,与NTDT相关的并发症可能很严重,而且许多并发症与慢性贫血直接相关。了解NTDT很重要,本综述将概述在决定是否对这些患者启动输血治疗以及就输血间隔和治疗持续时间进行适当规划时应考虑的因素。

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