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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: Primary plasmablastic lymphoma (PBL) is a rare and highly aggressive B cell lymphoma that occurs in the oral cavity of HIV-positive patients. This article reports the first HIV-negative primary vaginal stump PBL, and reviewed the literature review of the clinical and pathological features of PBL, diagnosis and differential diagnosis, treatment and prognosis. Methods: The clinical and pathological data of the case were retrospectively analyzed, and the related literature at home and abroad was discussed. Results: The diagnosis of this patient was clear, HIV negative, onset of vaginal stump, Immunohistochemistry showed MUM-1 positive, do not express CD20, CD79a and PAX-5, Ki-67 positive rate of 73%. EBER in situ hybridization negative. Molecular diagnostic results show: clonal rearrangement of sample immunoglobulin genes, clonally rearranged TCR gene was not detected. CHOP-like program ineffective, partially relieved the rapid progress of the disease. The time from diagnosis to death was 10.3 months. CONCLUSION: PBL is rare, the disease progresses rapidly, the prognosis is poor, and the life span is short. In the diagnosis and differential diagnosis of vaginal stump tumor, the PBL of vaginal stump should be included in the range of identification.