Park Sanghui, Ko Young H
Department of Pathology, Ewha Womans University School of Medicine, Seoul, Korea.
J Dermatol. 2014 Jan;41(1):29-39. doi: 10.1111/1346-8138.12322.
Primary infection with Epstein-Barr virus (EBV) is usually asymptomatic and, in a normal host, EBV remains latent in B cells after primary infection for the remainder of life. Uncommonly, EBV can infect T or natural killer (NK) cells in a person with a defect in innate immunity, and EBV infection can cause unique systemic lymphoproliferative diseases (LPD) of childhood. Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T-cell LPD of childhood. Uncommonly, patients can develop chronic active EBV (CAEBV) disease-type T/NK LPD, which includes CAEBV infection of the systemic form, hydroa vacciniforme-like T-cell LPD, and mosquito-bite hypersensitivity. The clinical course of CAEBV disease-type T/NK LPD can be smoldering, persistent or progressive, depending on the balance between viral factors and host immunity. Aggressive NK-cell leukemia, hydroa vacciniforme-like T-cell lymphoma, or uncommonly extranodal NK/T-cell lymphoma can develop in children and young adults with CAEBV disease-type T/NK-cell LPD. Extranodal T/NK-cell lymphoma is a disease of adults, and its incidence begins to increase in the third decade and comprises the major subtype of T/NK LPD throughout life. Aggressive NK-cell leukemia and nodal T/NK-cell lymphoma of the elderly are fulminant diseases, and immune senescence may be an important pathogenetic factor. This review describes the current progress in identifying different types of EBV-associated T/NK-cell LPD and includes a brief presentation of data from Korea.
爱泼斯坦-巴尔病毒（EBV）的初次感染通常没有症状，在正常宿主中，初次感染后EBV会在B细胞中终身潜伏。罕见的是，EBV可感染先天免疫存在缺陷者的T细胞或自然杀伤（NK）细胞，EBV感染可导致儿童独特的系统性淋巴增殖性疾病（LPD）。幼儿的初次感染可能并发噬血细胞性淋巴组织细胞增生症或儿童暴发性系统性T细胞LPD。罕见的是，患者可发展为慢性活动性EBV（CAEBV）病型T/NK LPD，包括全身性CAEBV感染、种痘样水疱病样T细胞LPD和蚊虫叮咬超敏反应。CAEBV病型T/NK LPD的临床病程可能是隐匿性、持续性或进行性的，这取决于病毒因素和宿主免疫之间的平衡。患有CAEBV病型T/NK细胞LPD的儿童和年轻人可能会发展为侵袭性NK细胞白血病、种痘样水疱病样T细胞淋巴瘤，或罕见的结外NK/T细胞淋巴瘤。结外T/NK细胞淋巴瘤是一种成人疾病，其发病率在第三个十年开始上升，并在一生中构成T/NK LPD的主要亚型。老年人的侵袭性NK细胞白血病和结内T/NK细胞淋巴瘤是暴发性疾病，免疫衰老可能是一个重要的致病因素。本综述描述了在识别不同类型的EBV相关T/NK细胞LPD方面的当前进展，并简要介绍了来自韩国的数据。
