单形性嗜上皮性肠道T细胞淋巴瘤3例报道与文献复习  

Monomorphic epitheliotropic intestinal T-cell lymphoma: A clinic-pathologic study of 3 cases and literature review

作　　者：王莉 黄德鸿 肖春燕 李昱[2] 张文军 郭冰凌 龚奕 杨涛 李启英 南映瑜 张军[3] 项颖 WANG Li;HUANG De-hong;XIAO Chun-yan;LI Yu;ZHANG Wen-jun;GUO Bing-lin;GONG Yi;YANG Tao;LI Qi-ying;NAN Yin-yu;ZHANG Jun;XIANG Ying(Department of Hematology and Ontology, Chongqing University Cancer Hospital ＆ Chongqing Cancer Institute ＆ Chongqing Cancer Hospital, Chongqing 400030, China;Department of Pathologyj Chongqing Medical University, Chongqing 400016, China;Department of Gastrointestinal Surgery, First Affiliated Hospital of Choncaing Medical University, Chanaaina 400016 China)

机构地区：[1]重庆大学附属肿瘤医院、重庆市肿瘤研究所、重庆市肿瘤医院血液肿瘤科,重庆400030 [2]重庆医科大学病理教研室,重庆400016 [3]重庆医科大学附属第一医院胃肠外科,重庆400016

出　　处：《解放军医学杂志》2018年第1期45-50,共6页Medical Journal of Chinese People's Liberation Army

基　　金：重庆市中青年医学高端后备人才培养项目(2017HBRC016)~~

摘　　要：目的结合病例报道和文献复习探讨单形性嗜上皮性肠道T细胞淋巴瘤(MEITL)的临床病理特征,为诊断和治疗提供经验和借鉴。方法回顾性分析3例MEITL患者的临床表现、组织病理学和免疫组化特点、诊治经过和预后,同时对既往发表文献中的其他案例进行复习和总结。结果本文报道的3例患者中,2例患者为中老年男性,1例为青年女性。3例患者的病变部位均在小肠,其中女性患者同时出现皮肤、乳腺受累。临床表现主要为腹胀、腹痛和肠道穿孔;病理特征主要为全层肠壁被大小均一的肿瘤细胞弥漫性浸润。免疫组化检测显示肿瘤细胞表达T细胞标记CD3、CD8、CD56、T细胞胞浆内抗原(TIA-1)、CD4和CD5;Ki-67增殖指数为50%~70%。在治疗上,病例1先后接受CHOP方案和GDP方案治疗,于确诊2年后死亡。病例2接受CHOP方案化疗1个疗程,随后放弃治疗死亡。病例3放弃治疗自动出院失访。结合既往发表的文献可见,MEITL发病率低,诊断较困难,具有高度侵袭性,患者对现有治疗的反应均较差。结论 MEITL好发于小肠,少见、进展快,患者易发生肠道穿孔、预后差,其诊断需结合临床表现、病理学特征和免疫组化检测结果等进行综合判断。尽早手术明确诊断并积极化疗可改善预后。Objective To summarize the clinicopathological features of monomorphic epitheliotropic intestinal T cell lymphoma（MEITL）, and provide the experiences and lessons for diagnosis and treatment. Methods The clinical manifestations, histopathological and immunohistochemical characteristics, diagnosis, treatment and prognosis of 3 cases of MEITL were analyzed retrospectively. Meanwhile, the other cases published in the literatures were reviewed and summarized. Results Two cases of middle aged and aged men and one young woman were reported in present paper. These lesions of all the 3 patients were at the small intestine, of which the female patients had skin and mammary gland involvement. The clinical manifestations were abdominal distention, mulligrubs and intestinal perforation, and the main pathological feature was the diffuse infiltration of the homogeneous tumor cells in the whole layer intestinal wall. Immunohistochemical examination showed that tumor cells expressed all T cell markers CD3, CD8, CD56, T cell cytoplasmic antigen（TIA-1）, CD4 and CD5, and Ki-67 proliferation index was 50%–70%. As to the treatment, case 1 received CHOP and GDP regimens successively, and died 2 years after final diagnosis. Case 2 received CHOP regimen for 1 course of chemotherapy and died of giving up treatment. Case 3 gave up treatment, voluntary discharged and then lost. It is clear according to the previous literatures and our experiences that the MEITL possesses high invasiveness and low incidence, and makes the diagnosis difficult; the patient＇s response to the current treatment is poor. Conclusions MEITL is prone to be in the small intestine. The diagnosis of MEITL should be based on clinical manifestations, pathological features and immunohistochemical detection results. MEITL is rare, the course of disease progresses rapidly, and the patients have the tendency to have intestinal perforation and poor prognosis. Early diagnosis and positive chemotherapy may lead to a better prognosis.

关 键 词：单形性嗜上皮性肠道T细胞淋巴瘤 诊断 治疗 

分 类 号：R733.4[医药卫生—肿瘤]