成人慢性活动性EB病毒感染的临床特点及预后分析

摘要/Abstract

摘要： 目的探讨成人慢性活动性EB病毒感染(chronic active Epstein-Barr virus infection, CAEBV)的临床特征,为临床诊治提供依据。方法回顾性分析2008年1月至2019年12月41例成人CAEBV患者的临床资料,包括起病方式、临床表现、实验室检查(EB病毒DNA及抗体谱、细胞因子、组织EBER等)、治疗和随访情况。结果 41例CAEBV患者中,男女比例为1.2∶1(男22例,女19例),中位年龄42岁(15～75岁)。间歇性发热38例(92.7%),伴有淋巴结肿大36例(87.8%),脾肿大35例(85.4%),肝脏肿大28例(68.3%),以消化系统、呼吸系统及血液系统受累最常见。危及生命的并发症包括噬血细胞综合征9例(21.9%)、肝衰竭5例(12.2%)、间质性肺炎致ARDS 4例(9.8%)、恶性淋巴瘤3例(7.3%)。41例CAEBV患者血清EBV DNA平均值为4.3×10⁴拷贝/mL。41例患者中11例(26.8%)确诊后3个月内死亡。死亡组与存活组患者的PLT计数分别为(53.3±29.2)×10⁹/L和(88.1±36.7)×10⁹/L,TBil分别为(141.4±78.5)μmol/L和(61.3±34.4)μmol/L,AST分别为(210.5±99.6)U/L和(78.8±89.2)U/L,LDH分别为(864.6±478.6)U/L和(445.2±337.5)U/L,铁蛋白分别为(1 933.9±166.7)ng/mL和(687.5±103.8)ng/mL,IL-2受体分别为(5 253.6 ± 736.4)U/L和(1 989.0±131.5)U/L,IL-10分别为(486.1±125.7)pg/mL和(151.2 ±232.5)pg/mL,差异均有统计学意义(t=3.055、-3.759、-3.542、-4.715、-6.672、-3.965和-3.452,均P<0.01)。结论成人CAEBV临床表现复杂、预后差,PLT减少、TBil、AST、LDH、铁蛋白升高,伴有细胞因子IL-2受体及IL-10水平增加可能是预后不良的潜在危险因素。

关键词: 慢性活动性EBV感染, EBV相关的噬血淋巴组织细胞增生症, 细胞因子, EBER原位杂交, 治疗

Abstract: Objective To investigat the clinical features of adult-onset chronic active Epstein-Barr virus infection(CAEBV)and to provide a basis for the diagnosis and treatment of CAEBV.Methods The clinical data of 41 adults with CAEBV from January 2008 to December 2019 was analyzed retrospectively, including onset, clinical manifestation, EBV DNA, EBV antibodies, cytokines, EBER in situ hybridization, treatment and follow-up results. Results Among the 41patients with CAEBV, the male to female ratio was 1.2∶1(22 males and 19 females), and the median age was 42 years(15-75 years). According to the analysis of the onset of the patients, 38 cases (92.7%), 36 cases (87.8%), 35 cases (85.4%) and 28 case (68.3%) of the patients presented intermittent fever, accompanied by lymph node enlargement, splenomegaly and hepatomegaly, with the most common involvement of the digestive, respiratory and blood systems. Life-threatening complications included 9 cases (21.9%) of hemophagocytic syndrome, 5 cases (12.2%) of liver failure, 4 cases (9.8%) of ARDS caused by interstitial pneumonia, and 3 cases (7.3%) of malignant lymphoma. The mean value of serum EBV DNA in 41 CAEBV patients was 104.3copy/ml. Eleven of the 41 cases (26.8%) died within 3 months. The platelet counts (53.3±29.2)×10⁹/L vs(88.1±36.7)×10⁹/L in death group was significantly lower than survival group (t=3.055, P ±0.01). At the same time serum TBil levels(141.4±78.5)μmol/L vs(61.3±34.4)μmol/L,AST(210.5±99.6)IU/L vs(78.8±89.2)IU/L,LDH(864.6±478.6)IU/L vs (445.2±337.5)IU/L,ferritin(1933.9±166.7)ng/mL vs(687.5±103.8)ng/mL, IL-2R(5 253.6± 736.4)U/L vs(1 989.0± 131.5)U/L,IL-10(486.1±125.7)pg/mL vs(151.2 ±232.5)pg/mL in death group were all significantly higher than survival group(t=-3.759、-3.542、-4.715、-6.672、-3.965 and -3.452,all P<0.01).Conclusion The study reveals that CAEBV in adult patients has a severe clinical course and prognosis is poor. Lower thrombocytopenia and higher TBil、AST、LDH、ferritin、IL-2R and IL-10 might potentially be risk factor for a poor prognosis. EBV loads should be measured and tissue should be stained on hybridization (EBER) if a patient presents with the known symptoms of CAEBV.

Key words: Chronic active Epstein-Barr virus infection(CAEBV), Hematophagous lymphocyte hyperplasia (EBV-HLH), Cytokines, EBER in situ hybridization

史冬梅, 刘芸野, 王伟静, 项晓刚. 成人慢性活动性EB病毒感染的临床特点及预后分析[J]. 肝脏, 2021, 26(2): 182-186.

SHI Dong-mei, LIU Yun-ye, WANG Wei-jing, XIANG Xiao-gang. Clinical features of adult patients with chronic active Epstein-Barr virus infection[J]. Chinese Hepatolgy, 2021, 26(2): 182-186.

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