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目的探讨子宫原发性血管内大B细胞淋巴瘤(primary uterine intravascular large B-cell lymphoma,PU-IVLBCL)的临床病理学特征、治疗及预后。方法收集1例PU-IVLBCL的临床病理资料,观察其形态学及免疫表型特征,并复习相关文献。结果患者女性,80岁,因子宫脱垂2年而行全子宫切除术,病理检查时偶然发现子宫多处小血管内异型淋巴瘤细胞聚集。免疫表型:肿瘤细胞CD45、CD20、CD79a、PAX-5、MUM1、CD5和BCL-2均弥漫阳性,Ki-67增殖指数为80%。结论 IVLBCL是一种罕见的高度侵袭性结外大B细胞淋巴瘤亚型,早期不易被发现。病理检查是确诊的金标准。PU-IVLBCL极其罕见,该肿瘤需与其他类型淋巴瘤、白血病、癌栓、血管源性肿瘤等进行鉴别;其预后差,部分病例于确诊后短期内死亡。
Objective To investigate the clinicopathological features, treatment and prognosis of primary uterine intravascular large B-cell lymphoma (PU-IVLBCL). Methods The clinical and pathological data of one case of PU-IVLBCL were collected and their morphological and immunophenotypic characteristics were observed. Relevant literatures were reviewed. Results The female patient, aged 80, underwent hysterectomy for 2 years due to uterine prolapse. Occasionally, multiple small intravascular endovascular lymphoma cells were found in the uterus during the pathological examination. Immunophenotype: The tumor cells were diffusely positive for CD45, CD20, CD79a, PAX-5, MUM1, CD5 and BCL-2 with a Ki-67 proliferation index of 80%. Conclusion IVLBCL is a rare subtype of highly invasive extra-large B-cell lymphoma that is not easily detected early. Pathological examination is the gold standard confirmed. PU-IVLBCL is extremely rare. The tumor needs to be differentiated from other types of lymphoma, leukemia, thrombus and angiogenic tumor. The prognosis is poor. Some patients died shortly after diagnosis.