Acquired pure red cell aplasia: unraveling the immune pathogenesis

Mengyuan Liu, Tian Zhang, Xifeng Dong, Huaquan Wang
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Abstract

Acquired pure red cell aplasia (aPRCA) is a rare hematological disorder characterized by normochromic, normocytic anemia, reticulocytopenia, and the absence of erythroblasts. The pathogenesis of aPRCA has remained elusive. This review delves into the intricate web of immune mechanisms underlying the development of this enigmatic condition. By exploring immune responses, cytotoxic effects, and antibody-mediated processes, we dissect the immune-driven assault on erythroid progenitors. The classification of aPRCA, including its primary and secondary forms, is elucidated, with a particular emphasis on etiological factors such as viruses, drugs, thymoma, and large granular lymphocytic leukemia. Furthermore, we discuss the implications of cytogenetic changes in erythroid progenitors and immune cells in the pathophysiology of aPRCA. This comprehensive overview aims to shed light on the complex interplay between immune dysregulation and erythroid failure in aPRCA, offering insights that will be crucial for better understanding and treating this disease.
获得性纯红细胞再生不良症:揭示免疫发病机制
获得性纯红细胞增生症(aPRCA)是一种罕见的血液病,以正常色素、正常红细胞性贫血、网状细胞减少和红细胞缺失为特征。aPRCA 的发病机制一直难以捉摸。本综述将深入探讨这一神秘病症发病背后错综复杂的免疫机制。通过探讨免疫反应、细胞毒性效应和抗体介导的过程,我们剖析了免疫驱动的对红细胞祖细胞的攻击。我们阐明了 aPRCA 的分类,包括其原发性和继发性形式,并特别强调了病毒、药物、胸腺瘤和大颗粒淋巴细胞白血病等病因。此外,我们还讨论了红细胞祖细胞和免疫细胞的细胞遗传学变化对 aPRCA 病理生理学的影响。本综述旨在揭示 aPRCA 中免疫失调与红细胞功能衰竭之间复杂的相互作用,为更好地理解和治疗这种疾病提供重要见解。
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