Luo Ling, Wang Huanling, Fan Hongwei, Xie Jing, Qiu Zhifeng, Li Taisheng
Department of Infectious Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Medicine (Baltimore). 2018 Mar;97(9):e9854. doi: 10.1097/MD.0000000000009854.
Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected.There were 28 adult onset CAEBV patients. The median age was 45 (range, 20-81). Most of the patients presented with fever; splenomegaly; lymphadenopathy and hepatitis. Unlike pediatric cases reported, the manifestations of cardiovascular diseases in our patients were pulmonary arterial hypertension, decreased cardiac function and aorta vasculitis. Prevalence of interstitial pneumonitis in our patients were comparatively higher and prevalence of hypersensitivity to mosquito bites were comparatively lower than that reported by Japan. In this study, CAEBV patients had decreased B cell, NK cell, CD4 cell and CD8 cell counts. The prevalence of low level of B cells, NK cells, CD4 cells was relatively higher than reported ever.Chinese adult onset CAEBV patients have different clinical characteristics and are featured by an immunosuppression status as demonstrated by decreased B cell, NK cell, CD4 cell and CD8 cell.
慢性活动性EB病毒（CAEBV）感染是一种罕见病，死亡率高。大多数CAEBV患者来自日本，且多为儿童病例。本研究旨在描述成人发病的CAEBV患者的临床特征及外周血淋巴细胞的免疫表型特征。我们回顾性分析了2012年至2016年期间北京协和医院收治的所有成人发病的CAEBV病例。收集了患者的人口统计学、临床、实验室数据以及外周血淋巴细胞的免疫表型数据。共有28例成人发病的CAEBV患者。中位年龄为45岁（范围20 - 81岁）。大多数患者表现为发热、脾肿大、淋巴结病和肝炎。与报道的儿童病例不同，我们患者的心血管疾病表现为肺动脉高压、心功能下降和主动脉血管炎。我们患者间质性肺炎的患病率相对较高，蚊虫叮咬过敏的患病率相对低于日本报道的数据。在本研究中，CAEBV患者的B细胞、NK细胞、CD4细胞和CD8细胞计数均减少。B细胞、NK细胞、CD4细胞低水平的患病率相对高于以往报道。中国成人发病的CAEBV患者具有不同的临床特征，其特点是存在免疫抑制状态，表现为B细胞、NK细胞、CD4细胞和CD8细胞减少。
