Paediatric Infectious Diseases Research Centre, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran.
Br J Haematol. 2013 Sep;162(5):573-86. doi: 10.1111/bjh.12422. Epub 2013 Jun 11.
Epstein-Barr virus (EBV), a ubiquitous human herpesvirus, maintains lifelong subclinical persistent infections in humans. In the circulation, EBV primarily infects the B cells, and protective immunity is mediated by EBV-specific cytotoxic T cells (CTLs) and natural killer (NK) cells. However, EBV has been linked to several devastating diseases, such as haemophagocytic lymphohistiocytosis (HLH) and lymphoproliferative diseases in the immunocompromised host. Some types of primary immunodeficiencies (PIDs) are characterized by the development of EBV-associated complications as their predominant clinical feature. The study of such genetic diseases presents an ideal opportunity for a better understanding of the biology of the immune responses against EBV. Here, we summarize the range of PIDs that are predisposed to EBV-associated haematological diseases, describing their clinical picture and pathogenetic mechanisms.
EB 病毒（EBV）是一种普遍存在的人类疱疹病毒，在人类中维持终生亚临床持续感染。在循环中，EBV 主要感染 B 细胞，而 EBV 特异性细胞毒性 T 细胞（CTL）和自然杀伤（NK）细胞介导保护性免疫。然而，EBV 与几种毁灭性疾病有关，如噬血细胞性淋巴组织细胞增生症（HLH）和免疫功能低下宿主中的淋巴组织增生性疾病。某些类型的原发性免疫缺陷（PID）的特征是发展为 EBV 相关并发症作为其主要临床特征。对这些遗传疾病的研究为更好地了解针对 EBV 的免疫反应生物学提供了一个理想的机会。在这里，我们总结了易发生 EBV 相关血液疾病的 PID 范围，描述了它们的临床表现和发病机制。
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