获得性纯红细胞发育不全。

Hideyuki Nakazawa
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引用次数: 0

摘要

获得性纯红细胞再生不全(PRCA)是一种主要发生于老年人的骨髓衰竭综合征,以骨髓贫血、网状红细胞减少和红细胞发育不全为特征。潜在的t细胞失调,通常与克隆性和/或STAT3基因突变相关,是在大多数疾病亚型中使用环孢素和其他定向免疫抑制疗法的基本原理,即胸腺瘤相关的PRCA,大颗粒淋巴细胞白血病相关的PRCA和特发性PRCA。尽管流行病学上罕见的PRCA排除了比较性临床试验,无法证明一种免疫抑制剂优于另一种,但一些回顾性研究表明,维持治疗对于避免输血依赖具有重要意义,而输血依赖可能导致PRCA患者预后较差。本综述主要根据最近更新的临床指南讨论了PRCA的临床方面。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Acquired pure red cell aplasia].

Acquired pure red cell aplasia (PRCA), characterized by anemia, reticulocytopenia, and erythroid hypoplasia in the marrow, is a bone marrow failure syndrome that mainly affects elderly adults. Underlying T-cell dysregulations, often associated with clonality and/or STAT3 gene mutation, have been a rationale for using cyclosporin and other directed immunosuppressive therapies in most of the disease subtypes, namely, thymoma-associated PRCA, large granular lymphocytic leukemia-associated PRCA, and idiopathic PRCA. Although the epidemiologic rarity of PRCA has precluded comparative clinical trials that could demonstrate the superiority of one immunosuppressive agent over another, some retrospective studies have demonstrated the significance of maintenance therapies to avoid blood transfusion dependency, a factor that may lead to poorer prognosis in patients with PRCA. This review primarily discusses clinical aspects of PRCA in line with recently updated clinical guidelines.

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