人类免疫缺陷病毒阴性的阴道残端浆母细胞淋巴瘤一例并文献复习  

A Case of Plasmablastic Lymphoma of the Vagina Stump without Human Immunodeficiency Virus Infection and Literature Review

作　　者：文禄 郭英[2] 谢云霞[1] 范国庆[2] 陈协群[1] 杨岚[1] 

机构地区：[1]第四军医大学西京医院血液内科,陕西西安710032 [2]第四军医大学西京医院病理科,陕西西安710032

出　　处：《现代生物医学进展》2014年第20期3860-3862,3872,共4页Progress in Modern Biomedicine

基　　金：国家自然科学基金项目(81071951);陕西省科技攻关项目(2010K01-163)

摘　　要：目的:浆母细胞淋巴瘤(PBL)为罕见的、侵袭性极强的B细胞淋巴瘤,好发于HIV阳性患者的口腔,本文报道了目前国内外首例HIV阴性的原发于阴道残端的PBL,并通过文献复习总结了PBL的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法:回顾分析该病例的临床病理学资料,并结合国内外相关文献进行讨论。结果:该患者诊断明确,HIV阴性,以阴道残端起病,免疫组化示MUM-1阳性,不表达CD20、CD79a和PAX-5,Ki-67阳性率73%。EBER原位杂交阴性。分子学诊断结果示:样品免疫球蛋白基因发生克隆性重排,未检到TCR基因克隆性重排。CHOP样方案疗效不佳,部分缓解后疾病快速进展。该患者从确诊至死亡时间为10.3个月。结论:PBL罕见,病情进展迅速,预后差,生存期短,在进行阴道残端肿瘤的诊断及鉴别诊断时,阴道残端PBL应纳入鉴别范围。Objective： Plasmablastic lymphoma （PBL） was a rare, aggressive variant of B-cell lymphoma that frequently occurred in the oral cavity of patients with human immunodeficiency virus （HIV） infection. We reported the first case ofHIV-negative PBL with initial presentation of the vagina stump to investigate the clinicopathological characteristics, diagnosis, differential diagnosis, treatment and prognosis of PBL. Methods： The relevant clinicopathological materials of PBL patient was retrospected and a literature review was conducted. Results： The patient was explicitly diagnosed to be H1V negative with a vaginal stump mass as the main lesion. Immunohistochemical examination revealed that the tumor cells were positive for MUM-l, negative for CD20, CD79a and PAX-5. The Ki-67 proliferative index was 73%. The tumor cells were negative for EBV virus-encoded RNA in situ hybridization （EBER-ISH）. Molecular diagnostic results showed immunoglobulin gene rearrangement in the sample analyzed. TCR gene rearrangement was not observed. CHOP-likeregime showed no satisfactory effect and the disease progressed soon after a partial response. The patient died 10.3 months after the diagnosis. Conclusions： As PBL was a rare distinct variant with high malignancy, poor prognosis and shorter overall survival, it should be included as a differential diagnosis in cases of suspected vaginal stump tumor.

关 键 词：浆母细胞淋巴瘤 人类免疫缺陷病毒 阴道残端 

分 类 号：R733[医药卫生—肿瘤]