原发性免疫缺陷的eb病毒感染

IF 0.3 Q4 IMMUNOLOGY
A. Ovadia, I. Dalal
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引用次数: 2

摘要

原发性免疫缺陷(PID)是一组影响免疫细胞发育、分化和功能的遗传性疾病。受影响的个体极易受到多种病原体的感染。爱泼斯坦-巴尔病毒(EBV)感染在人类中普遍存在,通常涉及无症状或自限性临床过程。在极少数情况下,EBV不仅可以引起急性感染,还可以引起严重的过度免疫反应和淋巴细胞增生性疾病。此外,除了急性感染的严重过程外,PID患者的EBV感染可导致免疫失调和恶性肿瘤风险增加。识别不同的遗传缺陷及其对免疫途径的影响为我们提供了对EBV感染和相关疾病的病理生理学的基本见解,并可能导致未来开发更好的靶向治疗。在这里,我们回顾了所有对EBV疾病有异常反应的pid。新奇声明:在这里,我们回顾了目前已知的与EBV感染和相关疾病(如噬血细胞性淋巴组织细胞病)异常反应相关的所有pid。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Epstein–Barr virus infection in primary immunodeficiency
Primary immunodeficiency (PID) is a group of genetic disorders which affects immune cell development, differentiation, and function. The affected individuals are highly susceptible to infection by a diverse array of pathogens. Epstein–Barr virus (EBV) infection is ubiquitous in humans and usually involves an asymptomatic or self-limiting clinical course. In rare cases, EBV can cause not only an acute infection but also a severe exaggerated immune response and lymphoproliferative disease. Furthermore, EBV infection in patients with PID can lead to immune dysregulation and increased risk of malignancies, in addition to the severe course of the acute infection. Recognition of the different genetic defects and their effect on immunological pathways provide us with fundamental insights into the pathophysiology of EBV infection and associated disease, and may lead to developing better targeted therapies in the future. Here, we review all of PIDs with an abnormal response to EBV disease. Statement of novelty: Here we provide a review of the current knowledge of all PIDs reported to be associated with abnormal response to EBV infection and associated disease, such as hemophagocytic lymphohistiocytosis.
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