小肠浆母细胞淋巴瘤临床病理观察

Clinicopathologic features of plasmablastic lymphoma in small intestine

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 plasmablastic lymphoma （PBL） of the small intestine. Methods A case of PBL of the small intestine was analyzed by light microscopy, immunohistochemistry and in situ hybridization with review of the literature. Results The patient was a 55-year-old man. An irregular tumor nodule of the small intestine was excised. Histologically, the whole intestinal wall was diffusely infiltrated by malignant lymphoid cells with abundant basophilic cytoplasm, eccentrically located pleomorphic nuclei, and single, centrally located prominent nucleoli and the neoplastic cells also involved nearby multiple organs. lmmunohistochemically, the neoplastic cells were diffusely positive for CD79a, CD138 and CD10 and partly positive for CD38 and EMA. Nuclear proliferation rate as assessed by Ki-67 staining was approximately 80%. The ceils showed kappa light chain restriction. The patient died of multiple organ failure due to further deterioration 1.5 months after operation. Conclusions PBL of the small intestine is a rare entity of non-Hodgkin B cell lymphoma, with worse outcome and shorter survival period. Because HIV and EBV infection are not detected in the patient, an HBV infection and the history of radiotherapy probably lead to the iatrogenie immunocompromised state.