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目的探讨小肠浆母细胞淋巴瘤(PBL)的临床病理学特征及鉴别诊断。方法对1例小肠浆母细胞淋巴瘤进行光镜、免疫组化及原位杂交检测,并复习相关文献。结果患者男性,55岁。手术切除小肠不规则结节状肿物。镜下见浆细胞样分化的异型淋巴细胞弥漫浸润小肠肠壁,并累及周围多个器官,瘤细胞胞质丰富,胞核偏位,核仁大而明显,核分裂象易见。免疫组化示CD79a、CD138和CD10弥漫(+),CD38和EMA局灶(+),Ki-67阳性指数80%。κ轻链基因单克隆性重排。术后肿瘤复发1个半月死亡。结论小肠PBL是一种罕见的B细胞来源、高侵袭性非霍奇金淋巴瘤,预后差,生存期短。本例PBL人类免疫缺陷病毒和EB病毒检测阴性,因此其发生可能与乙型肝炎病毒感染及放射治疗导致的免疫功能低下有关。
Objective To investigate the clinicopathological features and differential diagnosis of small cell plasmapulmonary lymphoma (PBL). Methods One case of small intestinal plasma cell lymphoma was examined by light microscopy, immunohistochemistry and in situ hybridization. The related literatures were reviewed. Results The patient was male, 55 years old. Surgical removal of small intestine irregular nodular tumor. Microscopically see plasmacytoid differentiation of atypical lymphocytes infiltrating the small intestine, and involving multiple organs around the rich cytoplasm tumor cells, nuclear deviation, large and obvious nucleolus, mitotic easy to see. Immunohistochemistry showed CD79a, CD138 and CD10 diffuse (+), CD38 and EMA focal (+), Ki-67 positive index of 80%. Kappa light chain gene monoclonal rearrangement. Postoperative tumor recurrence 1 and a half months died. Conclusions Small intestinal PBL is a rare B cell source, highly aggressive non-Hodgkin’s lymphoma with poor prognosis and short survival. This case of PBL negative for human immunodeficiency virus and Epstein-Barr virus, so its occurrence may be associated with hepatitis B virus infection and radiation therapy-induced immune dysfunction.