摘要
目的探讨人免疫缺陷病毒（HIV）阴性的浆母细胞性淋巴瘤（PBL）的临床病理特征、免疫表型、分子遗传学特点及预后。方法回顾性分析2005至2014年间的可疑为PBL的病例，根据WHO（2008）造血与淋巴组织肿瘤新分类中PBL诊断标准，确诊并总结12例HIV阴性的PBL的临床病理特点、免疫表型，随访获取患者的预后信息，并复习相关文献。结果患者多为男性（11／12），中位年龄55．5岁，肿瘤细胞呈免疫母细胞或浆母细胞样特点，免疫组织化学表达浆细胞标记，不表达成熟B细胞标记，10／12的病例Ki-67阳性指数≥80％，7／10的病例EB病毒编码的小RNA（EBER）阳性。可伴有C-myc基因易位重排（2／5）。12例PBL患者中4例死亡。结论PBL是一类罕见的、高侵袭性的B细胞淋巴瘤，而HIV阴性的PBL则更为少见，侵犯口腔及EB病毒阳性的概率低于HIV阳性者，鉴别诊断存在很大挑战，预后更差。
Objective To study the clinical, pathologic, immunophenotype, molecular characteristics and prognosis of HIV-negative plasmablastic lymphoma （PBL）. Methods Twelve cases of HIV-negative PBLs diagnosed between 2005 and 2014 in Guangdong General Hospital were identified according to WHO classification of tumors of haematopoietie and lymphoid tissues （2008）. The clinicopathologic features and outcome were analyzed and the relevant literatures were reviewed. Results The patients were predominantly male （ 11/12 ） with a median age of 55.5 years. The tumor cells showed the characteristic combination of immunoblastic/plasmablastic morphology, plasma cell phenotype and high proliferation, no expression of mature B cell markers. 7/10 of the cases were EBER positive. Two cases were positive for C-myc translocation. Four of twelve patients were died. Conclusions PBL is a rare, aggressive B-cell lymphoma. HIV-negative PBL has lower rate of oral involvement and EBER expression than HIV- positive patients, the differential diagnosis is very challenging, and the prognosis is worse.
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2015年第8期548-552,共5页
Chinese Journal of Pathology
基金
国家自然科学基金（81172244）
国家临床重点专科建设项目资助
关键词
淋巴瘤
疱疹病毒4型
人
诊断
鉴别
预后
Lymphoma
Herpesvirus 4,human
Diagnosis,differential
Prognosis
