Abstract:
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.
摘要:
EB病毒(EBV)是一种普遍存在的主要B细胞嗜性病毒。感染人类最常见的病毒之一,EBV,最著名的是传染性单核细胞增多症(IM)的病原体。虽然大多数人经历无症状感染,EBV是一种有效的免疫刺激物,因此它引起其感染的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。在某些个人中,比如那些有影响免疫系统的遗传或获得性缺陷的人,未能正确控制EBV导致EBV感染的B细胞和EBV反应性免疫细胞的积累,它们共同促进了经常危及生命的细胞因子风暴综合征(CSS)的发展。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,噬血细胞淋巴组织细胞增生症,移植后淋巴增生性疾病.鉴于细胞因子在驱动炎症和促进疾病发病机制中的关键作用,我们还讨论了靶向特定细胞因子如何为EBV驱动的CSS提供合理且潜在毒性较低的治疗。