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。

abstractsObjective 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 CD3ε;the median Ki-67 index was 80%.Five cases were EBER1/2 in situ hybridization positive.IgH or/and Igκgene rearrangement was detected in all five cases examined.Conclusions Most patients were no congenital or acquired immunodeficiency 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.