CAJ | 학술논문

目的探讨人类免疫缺陷病毒(HIV)阴性且无免疫缺陷的浆母细胞淋巴瘤(PBL)的临床病理特征,提高对这组疾患的认识.方法回顾性分析6例无免疫缺陷且HIV-PBL的组织学特点,原位杂交染色检测EB病毒(EBV)感染状态.分别采用免疫组织化学SP法及荧光原位杂交(FISH)技术检测PBL的免疫表型、EBV潜伏类型,探索myc基因的易位.结果 HIV-PBL表现为浆母细胞样或免疫母细胞样细胞的单一增生,可见瘤巨细胞及坏死;背景反应细胞少,核分裂象较多.所有病例都有EBV感染,潜伏类型为Ⅰ型(LMP1-及EBNA2-).肿瘤细胞表达B细胞终末分化阶段的表型CD20-/CD3-/CD138+/Kappa+或Lambda+.6例HIV-PBL均为老年患者(中位年龄69.5岁),男女各3例;结外及口腔外侵犯率高,分别为6、5例.中位生存期为25.5个月.此外,3例患者具有免疫球蛋白重链(IgH)与myc基因易位.结论 HIV-PBL是一组独立疾患,具有无HIV感染、老年人、EBV阳性、结外及口腔外侵犯率高等特点,应与HIV+的PBL相区别.
목적 탐토인류면역결함병독(HIV)음성차무면역결함적장모세포림파류(PBL)적림상병리특정,제고대저조질환적인식.방법 회고성분석6례무면역결함차HIV-PBL적조직학특점,원위잡교염색검측EB병독(EBV)감염상태.분별채용면역조직화학SP법급형광원위잡교(FISH)기술검측PBL적면역표형、EBV잠복류형,탐색myc기인적역위.결과 HIV-PBL표현위장모세포양혹면역모세포양세포적단일증생,가견류거세포급배사;배경반응세포소,핵분렬상교다.소유병례도유EBV감염,잠복류형위Ⅰ형(LMP1-급EBNA2-).종류세포표체B세포종말분화계단적표형CD20-/CD3-/CD138+/Kappa+혹Lambda+.6례HIV-PBL균위노년환자(중위년령69.5세),남녀각3례;결외급구강외침범솔고,분별위6、5례.중위생존기위25.5개월.차외,3례환자구유면역구단백중련(IgH)여myc기인역위.결론 HIV-PBL시일조독립질환,구유무HIV감염、노년인、EBV양성、결외급구강외침범솔고등특점,응여HIV+적PBL상구별.
Objective To investigate the clinicopathological features of human immunodeficiency virus (HIV) negative plasmablastic lymphoma (PBL) with no-immunosuppression,so as to accelerate the understanding for this group of disease.Methods The histological features of 6 HIV-PBL patients with no-immunodeficiency were retrospectively analyzed.Epstein-Barr virus (EBV) status was detected by in situ hybridization.Then,immunohistochemistry and fluorescence in situ hybridization (FISH) method were used to determine the immunophenotype,latent status of EBV and MYC translocation in PBL,respectively.Results HIV-PBL showed monotonous proliferation of plasmablastoid or immunoblast-like cells.Giant cells and necrosis could be observed,with less reactive cells in the background and higher mitoses.All the cases had EBV infection and type Ⅰ latency status of EBV (LMP1-/EBNA2-),and expressed terminal B-cell differentiation immunophenotype (CD20-/CD3-/CD138+/Kappa or Lambda+).Six HIV-PBL patients were elderly (median age was 69.5 years old),had equal incidence of PBL between male and female and showed high frequency of involvement of extranodal and extraoral lesion sites (6 cases and 5 cases,respectively).Median sutvival was 25.5 months.In addition,3 HIV-PBL patients had IGH/MYC translocations.Conclusions HIV-PBL is a new entity with unique clinical features including no-HIV infection,elderly,EBV positivity,and more involvement in extranodal and extraoral sites.HIV-PBL should be distinguished from HIV+ PBL.