Chronic Active Epstein-Barr Virus Infection: Is It Immunodeficiency, Malignancy, or Both?
Chronic Active Epstein-Barr Virus Infection: Is It Immunodeficiency, Malignancy, or Both?
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DOI：
10.3390/cancers12113202
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发表时间：
2020-10-30
期刊：
影响因子：
5.2
通讯作者：
Nakamura H
中科院分区：
文献类型：
--
作者：
Fujiwara S;Nakamura H
慢性活动性EB病毒（EBV）感染（CAEBV）是一种病因不明的罕见综合征，其特征在于长时间的传染性单核细胞增多症样症状和EBV感染的T细胞和/或自然杀伤细胞的增殖。CAEBV主要在东亚和拉丁美洲报道，表明其发病机制中存在遗传易感性。CAEBV的临床过程是异质性的，从惰性和偶尔自限性疾病到侵袭性和致命性疾病，但其预后通常较差。这种异质性的临床表现并不能提示该综合征的简单病因。临床病理学研究提示CAEBV具有恶性肿瘤和免疫缺陷的双重特征。本文总结了CAEBV的最新发现，并讨论了其发病机制和疾病概念的关键未解决的问题。慢性活动性EB病毒感染（Chronic active Epstein-Barr virus infection，CAEBV）是一种罕见的临床综合征，其特征是免疫功能正常者外周血EB病毒DNA载量明显升高，并伴有持续的传染性单核细胞增多样症状。CAEBV主要在东亚和拉丁美洲报道，表明其发病机制中存在遗传易感性。在大多数CAEBV病例中，EBV诱导其不寻常的宿主细胞T或自然杀伤（NK）细胞增殖。CAEBV的临床过程是异质性的;一些患者表现出惰性过程，保持稳定状态多年，而另一些患者表现出侵袭性过程，由于噬血细胞性淋巴组织细胞增多症、多器官衰竭或进展为白血病/淋巴瘤而导致致命结局。CAEBV的发病机制尚不清楚，临床病理学研究表明，它具有恶性肿瘤和免疫缺陷的方面。最近对CAEBV患者的病毒和宿主基因组的遗传分析已经导致了一些发现，这些发现正在提高我们对这种综合征性质的理解。本文总结了CAEBV的最新发现，并讨论了其发病机制和疾病概念的关键未解决的问题。
Chronic active Epstein–Barr virus (EBV) infection (CAEBV) is a rare syndrome of unknown etiology characterized by prolonged infectious mononucleosis-like symptoms and proliferation of EBV-infected T and/or natural killer cells. CAEBV has been primarily reported in East Asia and Latin America, suggesting a genetic predisposition in its pathogenesis. The clinical course of CAEBV is heterogeneous ranging from an indolent and occasionally self-limiting disease to an aggressive and fatal condition, but its prognosis is generally poor. This heterogeneous clinical picture does not suggest a simple etiology for the syndrome. Clinicopathological investigations of CAEBV suggest that it has aspects of both malignant neoplasm and immunodeficiency. This article summarizes the latest findings on CAEBV and discusses critical unsolved questions regarding its pathogenesis and disease concept. Chronic active Epstein–Barr virus (EBV) infection (CAEBV) is a rare syndrome characterized by prolonged infectious mononucleosis-like symptoms and elevated peripheral blood EBV DNA load in apparently immunocompetent persons. CAEBV has been primarily reported in East Asia and Latin America, suggesting a genetic predisposition in its pathogenesis. In most cases of CAEBV, EBV induces proliferation of its unusual host cells, T or natural killer (NK) cells. The clinical course of CAEBV is heterogeneous; some patients show an indolent course, remaining in a stable condition for years, whereas others show an aggressive course with a fatal outcome due to hemophagocytic lymphohistiocytosis, multiple organ failure, or progression to leukemia/lymphoma. The pathogenesis of CAEBV is unclear and clinicopathological investigations suggest that it has aspects of both malignant neoplasm and immunodeficiency. Recent genetic analyses of both viral and host genomes in CAEBV patients have led to discoveries that are improving our understanding of the nature of this syndrome. This article summarizes the latest findings on CAEBV and discusses critical unsolved questions regarding its pathogenesis and disease concept.
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影响因子：
20.3
作者：
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通讯作者：
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影响因子：
11.8
作者：
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通讯作者：
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影响因子：
11.8
作者：
Cohen, Jeffrey I.;Dropulic, Lesia;Holland, Steven M.
通讯作者：
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影响因子：
20.3
作者：
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通讯作者：
Cohen, Jeffrey I.
DOI：
10.1084/jem.20160849
发表时间：
2017-01
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Abolhassani H;Edwards ES;Ikinciogullari A;Jing H;Borte S;Buggert M;Du L;Matsuda-Lennikov M;Romano R;Caridha R;Bade S;Zhang Y;Frederiksen J;Fang M;Bal SK;Haskologlu S;Dogu F;Tacyildiz N;Matthews HF;McElwee JJ;Gostick E;Price DA;Palendira U;Aghamohammadi A;Boisson B;Rezaei N;Karlsson AC;Lenardo MJ;Casanova JL;Hammarström L;Tangye SG;Su HC;Pan-Hammarström Q
通讯作者：
Pan-Hammarström Q