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

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。

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

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.