成人EB病毒阳性T/NK细胞淋巴组织增殖性疾病的临床病理特征、免疫表型和EBV感染特征分析

Analysis of Clinicopathological Features,Immunophenotyping and EBV Infection Characteristics of Lymphoid Tissue Proliferative Diseases in Adult EB Virus-positive T/NK Cells

目的探究成人EB病毒阳性T/NK细胞淋巴组织增殖性疾病(ASEBVT/NK-LDP)的临床病理特征、免疫表型和EBV感染特征。方法选取2014年1月—2017年12月北京大学深圳医院收治的ASEBVT/NK-LDP患者30例作为研究对象,统计所有患者的临床资料,病理学改变、免疫组织表型及EBV感染情况。结果临床症状主要为发热、淋巴结肿大及肝脾肿大,少数出现蚊虫叮咬超敏反应;实验室检测结果显示:患者肝功能异常、血沉加快。30例患者中17例患者死亡,生存时间为(2.97±1.62)个月,中位生存年龄为2个月;患者出现淋巴结被膜增厚且伴纤维化,被膜中出现大量小淋巴细胞及不规则的淋巴细胞浸润;淋巴结中出现淋巴滤泡,淋巴结构破坏,且呈现出病理状弥散。肝窦内及肝汇管区的淋巴细胞形状不规则且出现细胞浸润。红髓结果出现显著扩张,而白髓结构不清,淋巴细胞浸润明显。胃肠道黏膜出现糜烂、溃疡等情况。造血细胞间出现不规则、含有小灶状的淋巴细胞。CD4及CD8细胞比例有所增加,有10例患者T细胞以CD4阳性细胞为主,15例以CD8阳性细胞为主。免疫组化对患者组织切片进行CD5及CD7染色,10例患者出现CD5丢失,5例出现CD5及CD7丢失。EBV抗体检测结果显示,30例患者中21例出现阳性结果,7例出现多克隆性增生。结论 ASEBVT/NK-LDP患者临床表现为发热及肝脾肿大,病灶处活检显示组织损伤,并出现一定的细胞浸润,EVB感染的T细胞入侵器官导致患者死亡,一旦出现相应症状应及时进行ERBR原位杂交确诊,并采取积极治疗措施。

Objective To investigate the clinicopathological features, immunophenotype and EBV infection characteristics of adult EB virus-positive T/NK cell lymphoid tissue proliferative disease(ASEBVT/NK-LDP). Methods 30 patients with ASEBVT/ NK-LDP admitted to the hospital from January,2014 to December,2017 were enrolled. The clinical data,pathological changes, immunological phenotype and EBV infection were statistically analyzed. Results The main clinical symptoms were fever,lymphadenopathy and hepatosplenomegaly. A few mosquito-bite hypersensitivity reactions occurred. Laboratory tests showed abnormal liver function and accelerated erythrocyte sedimentation rate. 17 of the 30 patients died,with a survival time of(2.97±1.62)months and a median survival age of 2 months. The patient developed thickened lymph nodes and fibrosis,and a large number of small lymphocyte and irregular lymphocytes infiltrated in the capsule;lymphoid follicles appeared in the lymph nodes,the structure was destroyed, and the pathological dispersion was observed. The lymphocytes in the hepatic sinus and the hepatic duct area were irregular in shape and underwent cell infiltration. The red pulp results showed significant expansion and the white pulp structure was unclear,and lymphocytic infiltration was evident. Gastrointestinal mucosa was erosive and ulcerated,etc. Irregular and small-follicular lymphocytes appeared among hematopoietic cells. Gastrointestinal mucosa was erosive and ulcerated,etc. The proportion of CD4 and CD8 cells increased. In 10 patients,T cells were mainly CD4 positive cells,and 15 patients were mainly CD8 positive cells. Immunohistochemistry stained CD5 and CD7 on the tissue sections of patients,10 patients had CD5 loss,and 5 patients had CD5 and CD7 loss. EBV antibody test results showed that 21 of 30 patients had positive results,and 7 patients had polyclonal hyperplasia. Conclusion The clinical manifestations of ASEBVT/NK-LDP patients are fever and hepatosplenomegaly. Biopsy at the lesion shows tissue damage and certain cell infiltration. EVB-infected T cells cause death of the organ due to organ invasion. ERBR in situ hybridization should be confirmed in time,and active treatment measures should be taken.