浆母细胞性淋巴瘤11例临床病理分析及文献复习  

Plasmablastic lymphoma： a clinicopathologic analysis of 11 cases with review of literature

作　　者：李芬[1] 丁文双[1] 左卓[2] 耿宁[3] 杨槐[4] 刘秀兰[5] 王健超[1] 要文青[1] 刘卫平[1] 

机构地区：[1]四川大学华西医院病理科,成都510041 [2]四川省肿瘤医院病理科 [3]四川大学华西口腔医院病理科 [4]四川省遂宁市中心医院病理科 [5]四川省内江市第二人民医院病理科

出　　处：《中华病理学杂志》2016年第1期37-42,共6页Chinese Journal of Pathology

摘　　要：目的探讨浆母细胞性淋巴瘤（PBL）的临床病理特征及病理诊断。方法复习11例PBL患者临床病理资料并随访，结合国内外PBL文献报道，分析PBL与人免疫缺陷病毒（HIV）及EB病毒感染的关系。结果10例PBL患者血清HIV抗体均阴性。男8例，女3例，中位年龄57岁。结外累及10例，淋巴结1例。中线部位病例5例（鼻咽鼻窦3例，口腔2例）。根据组织学特点，6例为口腔黏膜型，5例为伴浆细胞分化型；11例瘤细胞表达CD138和MUM–1，均不表达CD20和CD3ε，Ki–67阳性指数为80%；EBER1/2原位杂交阳性5例。5例进行免疫球蛋白IgH/IgK基因重排分析，均检出克隆性重排。结论本组多数患者无先天性或获得性免疫缺陷，死亡病例多为EBER阴性、Ⅳ期；肿瘤细胞主要呈免疫母样/浆母细胞样、可见浆细胞样细胞，具有浆细胞肿瘤免疫表型；PBL与浆细胞肿瘤鉴别存在困难，需要更多病例积累。Objective To investigate the clinicpathologic features and diagnosis of plasmablastic lymphoma （PBL）. Methods Eleven cases of PBL were collected and followed up, with review of the literature. HIV and EBV status and their relationships with the tumor were specially compared as well. Results In the current cohort, 10 patients were serologically HIV negative; the male to female ratio was 8 to 3, and the median age was 57 years. Ten cases showed extranodal involvement and one case was nodal based. At presentation, five patients had mid-facial involvement, including sinonasal area （3 cases） and oral cavity （2 cases）. Histologically, six were PBL of oral mucosa type, and five were PBL with plasmacytic differentiation. In all cases, the neoplastic cells expressed CD138 and MUM-1, and were negative for CD20 and CD3e; the median Ki-67 index was 80%. Five cases were EBER1/2 in situ hybridization positive. IgH or/and IgK gene rearrangement was detected in all five cases examined. Conclusions Most patients were no congenital or acquired immunodeficieney in the retrospective study. Of the died patients, EBER1/2 in situ hybridization were negative and their disease staging were IV, The neoplastic cells were immunoblastic or plasmablastic, sometimes the plasmacytoid cell can be seen and the neoplastic cell had mature plasma cell phenotype, the pathologic diagnosis of the lymphoma is still controversial now. Differentiate with plasma cell neoplasm is difficult, it is necessary to accumulate more cases for advanced study and observation in the future.

关 键 词：淋巴瘤 免疫表型分型 疱疹病毒4型 人 感染 

分 类 号：R733.1[医药卫生—肿瘤]