文章摘要
文禄1 郭英2 谢云霞1 范国庆2 陈协群1 杨岚1△.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*[J].,2014,14(20):3860-3862
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 HumanImmunodeficiency Virus Infection and Literature Review*
  
DOI:
中文关键词: 浆母细胞淋巴瘤  人类免疫缺陷病毒  阴道残端
英文关键词: Plasmablastic lymphoma  Human immunodeficiency virus  Vagina stump
基金项目:国家自然科学基金项目(81071951);陕西省科技攻关项目(2010K01-163)
作者单位
文禄1 郭英2 谢云霞1 范国庆2 陈协群1 杨岚1△ 1 第四军医大学西京医院血液内科陕西西安7100322第四军医大学西京医院病理科陕西西安710032 
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中文摘要:
      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。
英文摘要:
      ABSTRACT 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 of HIV-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 HIV negative with a vaginal stump mass as the main lesion. Immunohistochemical examination revealed that the tumor cells were positive for MUM-1, 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-like regime showed no satisfactory effect and the disease progressed soon after a partial response. The patient died 10.3 months after the diagnosis.Conclusion: 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.
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