胸腔原发性渗出性淋巴瘤1例临床病理学及基因重排分析  

Primary effusion lymphoma in pleural: A study of cytology,histology,immunohistochemistry and gene rearrangement

作　　者：石丛艳[1] 杨守京[2] 方正清[2] 巨安丽[1] 杨莉[2] 付欣[2] 王映梅[2] 姜敏玲 

机构地区：[1]西北工业大学医院病理科,陕西西安710072 [2]第四军医大学西京医院病理科,陕西西安710032

出　　处：《现代肿瘤医学》2016年第20期3276-3280,共5页Journal of Modern Oncology

摘　　要：目的:探讨胸腔原发性渗出性淋巴瘤临床病理特点及病理鉴别诊断、免疫组化表型、基因重排,旨在加深临床和病理对此疾病的认识。方法:报道1例原发性渗出性淋巴瘤,对其进行临床病理学分析及文献复习。结果:1例原发性渗出性淋巴瘤患者临床表现为反复发作的胸水以及右侧胸膜结节样增厚。胸水细胞学和胸膜穿刺组织学特征均显示肿瘤细胞从大免疫母细胞或浆母细胞到明显间变的细胞形态变化。免疫组化:CD45、CD38、CD138、EMA、WT-1、Vimentin均为阳性;CR、TTF-1、CD3、CD20、EBV均为阴性。基因重排结果显示:IGH、IGK、IGL在BIOMED-2 PCR受体基因重排引物设计系统中相应位置发生克隆性重排。结论:原发性渗出性淋巴瘤是一种罕见的非霍奇金淋巴瘤类型,它不同寻常的生长方式为临床和病理诊断带来了困难。该肿瘤需与弥漫性大B细胞淋巴瘤(免疫母细胞变异型)和间变性大细胞淋巴瘤等鉴别。即使联合化疗其预后仍然十分差,选择合理和特异的治疗靶向药物是治疗成功的基础。Objective： To explore the clinicopathological charac te ris tic s,th e pathological differential d iagnosis,the immunohistochemistry expression and the gene rearrangement of primary effusion lymphoma in pleural. Methods ： One case of primary effusion lymphoma was reported,with review of relevant literature. Results： The c l in ic a l manifestations of a patient with the primary exudative lymphoma（ PEL） are the recurrent attacks of hydrothorax and the nodular thickening of the right pleura. The histological features of the cytological examination of hydrothorax and thoracentesis both showed the morphological change from the large immunoblast or plasmacytoid cell to anaplastic cell. The tumor was positive for CD45 , CD38 , CD138 , EMA, WT - 1 and vimentin, but negative for CR, TTF - 1, CD3 , CD20 and EBV. The results of gene rearrangement show the clonal gene rearrangement of IGH,IGK and IGL at the correspond-ing positions in the primer design of BIOMED -2 but no TCR receptor gene rearrangement. Conclusion ： The PEL is rare type of non - Hodgkin lymphoma. The unusual growth makes the clinical and pathologic diagnosis difficult. This kind of tumor should be distinguished from the diffuse large - B cell lymphoma（ immunoblast cytometaplasia） and an-aplastic large cell lymphoma. To date, the prognosis for patients with PEL has been poor even the combined chemo-therapy are applied. It is hopeful that optimal and specific targets for therapy can become the basis for greater thera-peutic success.

关 键 词：原发性渗出性淋巴瘤 细胞学 组织学 鉴别诊断 免疫组化 基因重排 

分 类 号：R733.4[医药卫生—肿瘤]