The understanding of Epstein-Barr virus associated lymphoproliferative disorder

近年来，有关EB病毒（ EBV ）相关淋巴组织增殖性疾病（ LPD ）的文章越来越多， EBV＋LPD被使用越来越广。然而，其中所指的含义却不尽相同，由此引发了理解的困惑和交流的障碍。为此，我们通过文献复习和实际病例观察对EBV＋LPD的概念进行梳理和澄清，并阐述我们对EBV＋LPD的理解和认识。总体而言，目前主流观点认为，EBV＋LPD是指EBV感染的一组具有谱系的淋巴组织疾病，其中包括增生性、交界性、肿瘤性疾病。根据这个概念，EBV＋LPD不包括传染性单核细胞增多症（ IM）和急性重症EBV感染（ EBV＋嗜血细胞综合征、爆发性IM、致死性IM、爆发性EBV＋T－LPD等），也不包括已经明确命名的EBV＋淋巴瘤（如结外NK／T细胞淋巴瘤、侵袭性NK细胞白血病、Burkitt淋巴瘤、霍奇金淋巴瘤等）。 EBV＋LPD目前包括：（1）EBV＋B细胞－LPD：淋巴瘤样肉芽肿、EBV＋免疫缺陷相关LPD、慢性活动性EBV感染（ CAEBV ）－B细胞型、老年性EBV＋B细胞－LPD等。（2） EBV＋T／NK细胞－LPD：CAEBV－T／NK细胞型、种痘样水疱病、蚊叮超敏反应等。根据EBV＋T／NK细胞－LPD的发展进程，通常可分为1级、2级和3级三个级别。第1级为增生性疾病，第2级为交界性疾病，第3级为肿瘤性疾病。 EBV＋LPD不同于单纯的增生性疾病（如IM）但又有重叠，也不同于典型的肿瘤性疾病（如NK／T 细胞淋巴瘤）但也有重叠。临床上提高警惕，尽早识别与EBV＋LPD相关的严重并发症发生的苗头，从而及时避免严重并发症的发生，以及在并发症发生后积极治疗挽救生命，应作为管控这类疾病更重要的任务。In recent years , there are increasing articles concerning Epstein-Barr virus associated lymphoproliferative disorder (EBV+LPD), and the name of EBV +LPD is used widely.However,the meaning of EBV+LPD used is not the same , which triggered confusion of the understanding and obstacles of the communication.In order to solve this problem.Literature was reviewed with combination of our cases to clarify the concept of EBV +LPD and to expound our understanding about it .In general, it is currently accepted that EBV +LPD refers to a spectrum of lymphoid tissue diseases with EBV infection , including hyperplasia , borderline lesions , and neoplastic diseases .According to this concept , EBV+LPD should not include infectious mononucleosis ( IM ) and severe acute EBV infection ( EBV +hemophagocytic lymphohistiocytosis, fatal IM, fulminant IM, fulminant T-cell LPD), and should not include the explicitly named EBV+lymphomas ( such as extranodal NK/T cell lymphoma , aggressive NK cell leukemia , Burkitt lymphoma, and Hodgkin lymphoma , etc.) either.EBV +LPD should currently include: ( 1 ) EBV +B cell-LPD:lymphomatoid granulomatosis , EBV +immunodeficiency related LPD , chronic active EBV infection-B cell type, senile EBV +LPD, etc.(2) EBV +T/NK cell-LPD:CAEBV-T/NK cell type, hydroa vacciniforme, hypersensitivity of mosquito bite, etc.In addition, EBV+LPD is classified, based on the disease process , pathological and molecular data , as 3 grades:grade1, hyperplasia ( polymorphic lesions with polyclonal cells ); grade 2, borderline ( polymorphic lesions with clonality ); grade 3, neoplasm (monomorphic lesions with clonality).There are overlaps between EBV +LPD and typical hyperplasia, as well as EBV+LPD and typical lymphomas .However , the most important tasks are clinical vigilance , early identification of potential severe complications , and treating the patients in a timely manner to avoid serious complications , as well as the active treatment to save lives when the complications happened .中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)12817-8214