成人慢性活动性EB病毒感染患者的临床、病理特征及预后  

Clinical and pathological characteristics as well as prognosis of adult patients with chronic active Epstein-Barr virus infection

作　　者：张雯洁[1] 张启科[1] 冯友繁[1] 刘凤磊[2] 侯金霞[3] 魏小芳 ZHANG Wen-jie;ZHANG Qi-ke;FENG You-fan;LIU Feng-lei;HOU Jin-xia;WEI Xiao-fang(Department of Hematology,Gansu Provincial Hospital,Lanzhou 730000,China;Department of Pathology,Gansu Provincial Hospital,Lanzhou 730000,China;Department of Laboratory Medicine,Gansu Provincial Hospital,Lanzhou 730000,China)

机构地区：[1]甘肃省人民医院血液科,甘肃兰州730000 [2]甘肃省人民医院病理科,甘肃兰州730000 [3]甘肃省人民医院检验科,甘肃兰州730000

出　　处：《中国感染控制杂志》2024年第9期1098-1105,共8页Chinese Journal of Infection Control

基　　金：甘肃省科技计划项目(22JR5RA660)。

摘　　要：目的研究成人慢性活动性EB病毒(EBV)感染患者的临床、病理特点及诊断、治疗方法和预后。方法回顾性收集甘肃省某医院2017年1月-2022年12月收治的8例成人慢性活动性EBV感染患者的临床及病理资料,分析其临床及组织病理特点、EBV相关检测结果,以及患者治疗、预后情况。结果8例慢性活动性EBV感染患者中,男性3例,女性5例;中位年龄21.5岁,自起病至确诊中位时间为7个月;以发热、血细胞(累及外周血两系或三系)减少、淋巴结及肝脾大为主要表现。血浆EBV核酸(DNA)定量均>1.0×103。EBV感染细胞分选结果显示,3例为T淋巴细胞感染,2例为NK细胞感染,3例为T淋巴细胞和NK细胞共同感染。8例患者骨髓细胞学检查均未见异型淋巴细胞,6例可见噬血细胞。流式细胞术(FCM)分型结果显示,8例患者均未检出异常细胞群,不表达髓细胞、B淋巴细胞、T淋巴细胞及NK细胞标记,T细胞受体(TCR)基因重排阳性率为37.5%(3例),病理组化显示大部分病例(6例,75.0%)表达CD3,部分病例表达CD4、CD8、CD56、TIA-1,EBER均为阳性。患者经治疗后生存率为50.0%(4例),随访时间为6~51个月,1年生存率为85.7%,中位生存时间为24个月。结论慢性活动性EBV感染患者临床表现多变,可出现致死性并发症,应尽早诊断,个体化治疗,降低患者病死率。Objective To study the clinical and pathological characteristics,as well as diagnosis,treatment methods and prognosis of adult patients with chronic active Epstein-Barr virus infection(CAEBVI).Methods Clinical and pathological data of 8 adult patients with CAEBVI admitted to a hospital in Gansu Province from January 2017 to December 2022 were collected retrospectively,clinical and histopathological characteristics,EBV-related test results,as well as treatment and prognosis of patients were analyzed.Results Among 8 CAEBVI patients,3 were males and 5 were females,with the median age of 21.5 years.The median time from onset to diagnosis of CAEBVI was 7 months.The main manifestations were fever,pancytopenia(involving two or three peripheral blood lines),as well as lymph node enlargement,hepatomegaly and splenomegaly.The quantifications of plasma EBV nucleic acid(DNA)were all>1.0×103.The sorting results of EBV infected cells showed that 3 cases were T lymphocytes infection,2 were NK cell infection,and 3 were co-infection of T lymphocytes and NK cells.Bone marrow cytological examination of 8 patients showed no atypical lymphocytes,while 6 patients showed hemophagocytic cells.Flow cytometey(FCM)typing results showed that no abnormal cell population was detected in all the 8 patients,and no myeloid,B lymphocyte,T lymphocyte and NK cell markers were expressed.The positive rate of T cell receptor(TCR)gene rearrangement was 37.5%(n=3).Histopathology showed that most cases(n=6,75.0%)expressed CD3,partial cases expressed CD4,CD8,CD56,TIA-1,and EBV encoded RNA(EBER),all were positive.The survival rate of patients after treatment was 50.0%(n=4),the follow-up time was 6-51 months,the 1-year survival rate was 85.7%,and the median survival time was 24 months.Conclusion CAEBVI is characterized by variable clinical manifestations that may lead to fatal complications.Early diagnosis and individualized treatment should be performed to reduce mortality of patients.

关 键 词：慢性活动性EB病毒感染 EB病毒 噬血细胞性淋巴组织细胞增生症 

分 类 号：R512.7[医药卫生—内科学]