EB病毒相关的人类免疫缺陷病毒阴性浆母细胞淋巴瘤的临床病理分析

Objective To investigate the clinicopathological characteristics of plasmablastic lymphoma (PBL) in patients who are human immunodeficiency virus (HIV) negative and without immunodeficiency, so as to improve the understanding of this group of diseases. Methods The histological features of 6 cases of PBL without immunodeficiency and HIV (-) were retrospectively analyzed, and the Epstein - Barr virus (EBV) infection status was detected by in situ hybridization staining. The immunophenotype of tumor cells and the latent type of EBV were detected by immunohistochemical staining (SP method) and fluorescence in situ hybridization (FISH) technique respectively, and the translocation of MYC gene was explored. The reported cases of PBL without immunodeficiency and HIV (-) EBV (+) in the literature were reviewed, and the cases in the literature and the 6 cases in this group were analyzed in combination with clinical data. Results HIV (-) PBL showed a single proliferation of plasmablast - like or immunoblast - like cells, with tumor giant cells and necrosis visible. There were few background reactive cells and more mitotic figures. All cases had EBV infection, and the latent type was type I (LMP1 and EBNA2 -). The tumor cells expressed the phenotype of the terminal differentiation stage of B cells (CD20 -/CD3 -/CD138 +/Kappa + or Lambda +). All 6 cases of HIV (-) PBL were elderly patients (male:female = 1:1; median age 69.5 years), with a high rate of extranodal and extraoral invasion (100% and 83.3% respectively). The median survival time was 25.5 months. In addition, 3 cases had immunoglobulin heavy chain (IgH) and MYC gene translocation. Conclusion HIV (-) PBL is a group of independent diseases, characterized by no HIV infection, age - related, EBV positive, high rate of extranodal and extraoral invasion, etc., and should be distinguished from HIV (+) PBL.

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，以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染色（SP法）及荧光原位杂交（FISH）技术检测肿瘤细胞的免疫表型、EBV潜伏类型，探索MYC基因的易位，并复习无免疫缺陷且HIV（-）EBV(+)的PBL文献报道病例，结合临床资料对文献中的病例及该组6例患者进行分析。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，核分裂像较多。所有病例都有EBV感染，潜伏类型为Ⅰ型（LMP1及EBNA2-）。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（男：女=1：1;中位年龄69.5岁)，结外及口腔外侵犯高(分别为100%，83.3%)。中位生存时间为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、年龄相关、EBV阳性、结外及口腔外侵犯率高等特点，应与HIV（+）的PBL相区别。