Objective: To investigate the clinicopathological features of EBV-positive T/NK cell lymphoproliferative diseases (EBV(+) T/NK-LPD). Methods: The clinical characteristics of 156 cases of EBV(+) T/NK-LPD were collected from August 2002 to March 2015 at Beijing Friendship Hospital, Capital Medical University. Immunohistochemical staining, EBER in situ hybridization and clonal analysis of TCR gene were performed. All patients were followed up. Results: There were 106 male and 50 female patients; patients' age ranged from 1 to 75 years (median 20 years). The course of the diseases before diagnosis ranged from 2 to 540 months (median 20 months). Fever was noted in 122 patients (78.2%), 108 patients had lymphadenopathy (69.2%), and 75 patients had hepatosplenomegaly (48.1%). Thirty-three cases were grade 1, 68 cases were grade 2, and 55 cases were grade 3. TCR gene arrangement analysis was performed in 45 cases, and 33 cases (73.3%) showed clonal rearrangement. The follow-up period ranged from 1-134 months, and 44 patients (28.2%) died. There was a trend of increased death rate associated with increasing grade (P>0.05). Conclusions: There are many types of EBV(+) T/NK-LPD, and they can be classified as systemic, relatively localized and localized. The prognosis should be based on a comprehensive analysis of pathology and clinical data. There is no significant correlation between morphological grade and mortality. An important goal of therapy is to prevent serious complications.
目的： 分析EB病毒(EBV)阳性T/NK细胞淋巴组织增殖性疾病(EBV(＋)T/NK－LPD)的临床病理学特征。 方法： 总结2002年8月至2015年3月北京友谊医院病理科156例EBV(＋)T/NK－LPD患者的临床特点，进行形态学分级、免疫组织化学染色、EB病毒编码的小RNA(EBER)原位杂交及对45例患者进行T细胞受体(TCR)基因克隆性分析，并随访所有患者。 结果： (1)156例患者中，男性106例，女性50例，年龄1～75岁，中位年龄20岁，病程2～540个月，中位病程20个月，伴有发热122例(78.2%)，淋巴结肿大108例(69.2%)，肝脾肿大75例(48.1%)。(2)形态学分级1级33例，2级68例，3级55例。45例做了TCR检测，其中33例(73.3%)检测到克隆性重排，均为2或3级病例。(3)随访时间1～134个月，死亡44例(28.2%)。死亡原因最多为多器官功能衰竭21例(47.7%)。随着分级增高，有死亡百分比增高的趋势，但分级间差异无统计学意义(P>0.05)。 结论： EBV(＋)T/NK－LPD病例种类多样，表现为系统性、相对局限性和局限性病变，应基于病理和临床参数的综合评估患者情况，形态学分级与病死率无显著相关性；一旦诊断该病，就要警惕和预防严重并发症的发生。.
Keywords: Herpesvirus 4, human; Killer cells, natural; Lymphoproliferative disorders.