Fas/FasL and Complement Activation are Associated with Chronic Active Epstein-Barr Virus Hepatitis

作者全名："Lin, Jing; Su, Miao-Fang; Zheng, Jiao-Long; Gu, Lei; Wu, Hai-Cong; Wu, Xia; Lin, Hai-Yan; Wu, Zhi-Xian; Li, Dong-Liang"

作者地址："[Lin, Jing; Su, Miao-Fang; Zheng, Jiao-Long; Wu, Hai-Cong; Wu, Xia; Lin, Hai-Yan; Wu, Zhi-Xian; Li, Dong-Liang] Fujian Med Univ, 900TH Hosp Joint Logist Support Force, Dept Hepatobiliary Med, Fuzong Clin Med Coll,PLA, Fuzhou, Fujian, Peoples R China; [Gu, Lei] Chongqing Med Univ, Dept Resp & Crit Care Med, Affiliated Hosp 1, Chongqing, Peoples R China"

通信作者："Li, DL (通讯作者)，Fujian Med Univ, 900TH Hosp Joint Logist Support Force, Dept Hepatobiliary Med, Fuzong Clin Med Coll,PLA, Fuzhou 350025, Fujian, Peoples R China."

来源：JOURNAL OF CLINICAL AND TRANSLATIONAL HEPATOLOGY

ESI学科分类：　

WOS号：WOS:001047508100006

JCR分区：Q2

影响因子：3.1

年份：2023

卷号：11

期号：3

开始页：540

结束页：549

文献类型：Article

关键词：Chronic active EB virus hepatitis; Fas/FasL; Complement

摘要："Background and Aims: Chronic active Epstein-Barr virus hepatitis (CAEBVH) is a rare and highly lethal disease characterized by hepatitis and hepatomegaly. This study aimed to investigate the clinicopathological features and pathogenic mechanisms of CAEBVH. Methods: Ten patients with confirmed Epstein-Barr virus hepatitis infection were enrolled. The clinicopathological characteristics of these patients were summarized and analyzed. Flow cytometry was utilized to detect peripheral blood immune cell phenotypes and whole exome sequencing was used to explore pathogenic genetic mechanisms. Lastly, immunohistochemical staining was employed to verify pathogenic mechanisms. Results: Clinical features observed in all Epstein-Barr virus hepatitis patients included fever (7/10), splenomegaly (10/10), hepatomegaly (9/10), abnormal liver function (8/10), and CD8+ T cell lymphopenia (6/7). Hematoxylin and eosin staining revealed lymphocytic infiltration in the liver. Positive Epstein-Barr virus-encoded small RNA in-situ hybridization (EBER-ISH) of lymphocytes of liver tissues was noted. Whole exome sequencing indicated that cytotoxic T lymphocytes and the complement system were involved. The expression of CD8, Fas, FasL, and Caspase-8 expression as well as apoptotic markers was enhanced in the Epstein-Barr virus hepatitis group relative to the controls (p<0.05). Lastly, Complement 1q and complement 3d expression, were higher in CAEBVH patients relative to controls (p<0.05). Conclusions: CAEBVH patients developed fever, hepatosplenomegaly, and lymphadenopathy. Histopathological changes were a diffuse lymphocytic sinusoidal infiltrate with EBER-ISH positivity. Fas/FasL and complement activation were involved in CAE-BVH patients."

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