Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Adv Exp Med Biol. 2024;1448:227-248. doi: 10.1007/978-3-031-59815-9_16.
Epstein-Barr virus (EBV) is a ubiquitous and predominantly B cell tropic virus. One of the most common viruses to infect humans, EBV, is best known as the causative agent of infectious mononucleosis (IM). Although most people experience asymptomatic infection, EBV is a potent immune stimulus and as such it elicits robust proliferation and activation of the B-lymphocytes it infects as well as the immune cells that respond to infection. In certain individuals, such as those with inherited or acquired defects affecting the immune system, failure to properly control EBV leads to the accumulation of EBV-infected B cells and EBV-reactive immune cells, which together contribute to the development of often life-threatening cytokine storm syndromes (CSS). Here, we review the normal immune response to EBV and discuss several CSS associated with EBV, such as chronic active EBV infection, hemophagocytic lymphohistiocytosis, and post-transplant lymphoproliferative disorder. Given the critical role for cytokines in driving inflammation and contributing to disease pathogenesis, we also discuss how targeting specific cytokines provides a rational and potentially less toxic treatment for EBV-driven CSS.
EB 病毒（EBV）是一种普遍存在且主要感染 B 细胞的病毒。作为最常见的人类感染病毒之一，EBV 是传染性单核细胞增多症（IM）的病原体。尽管大多数人无症状感染，但 EBV 是一种强烈的免疫刺激物，因此它会引起受感染的 B 淋巴细胞以及对感染作出反应的免疫细胞的强烈增殖和激活。在某些个体中，如那些存在影响免疫系统的遗传或获得性缺陷的个体，不能正确控制 EBV 会导致 EBV 感染的 B 细胞和 EBV 反应性免疫细胞的积累，这些细胞共同导致常常危及生命的细胞因子风暴综合征（CSS）的发生。在这里，我们回顾了对 EBV 的正常免疫反应，并讨论了几种与 EBV 相关的 CSS，如慢性活动性 EBV 感染、噬血细胞性淋巴组织细胞增多症和移植后淋巴组织增生性疾病。鉴于细胞因子在驱动炎症和导致疾病发病机制中的关键作用，我们还讨论了如何针对特定细胞因子为 EBV 驱动的 CSS 提供合理且潜在毒性更小的治疗方法。
