单形性嗜上皮性肠道T细胞淋巴瘤11例临床分析

Clinical Analysis of 11 Cases of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma

目的总结单形性嗜上皮性肠道T细胞淋巴瘤(monomorphic epitheliotropic intestinal T-cell lymphoma,MEITL)的临床特征、诊治过程及预后。方法回顾性分析2014年5月~2021年5月我院11例MEITL临床资料。男10例,女1例。年龄27~69岁,中位年龄58岁。病变位于小肠7例,结直肠1例,同时累及小肠和结肠1例,小肠和食管1例,结肠和肺1例。8例经手术确诊,手术指征为肠穿孔5例,肠梗阻1例,小肠占位1例,食管占位1例,行小肠部分切除吻合术5例,小肠肿瘤切除、远端旷置、近端造瘘术1例,腹腔镜小肠部分切除吻合术1例,食管癌根治术1例,术后均接受化疗,术后首周期化疗均未发生严重的腹腔感染或手术伤口感染。3例内镜病理确诊,接受化疗,其中1例接受自体造血干细胞移植,1例化疗中肠梗阻行小肠部分切除吻合。结果10例死亡,中位生存期14.5月(3~25月),1例随访70个月生存。结论MEITL起病隐匿,常以急腹症为首诊症状,治疗以化疗为主,外科手术在辅助明确诊断及肠道并发症的治疗方面具有重要作用。

Objective To summarize the clinical features,diagnostic approach,treatment and prognosis of monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL).Methods Clinical and follow-up data of 11 cases of MEITL in our hospital from May 2014 to May 2021 were retrospectively analyzed.Ten cases were male and one case was female.The median age was 58 years old(range,27-69 years old).The lesions occurred in small intestine in 7 cases,colorectum in 1 case,small intestine and colon in 1 case,small intestine and esophagus in 1 case,and colon and lung in 1 case.Eight cases were diagnosed by surgical pathology,with operative indications of 5 cases of intestinal perforation,1 case of intestinal obstruction,1 case of intestinal mass,and 1 case of esophageal mass.The operations included 5 cases of intestinal resection and anastomosis,1 case of intestinal resection and jejunostomy,1 case of laparoscopic intestinal resection and anastomosis,and 1 case of radical resection of esophageal carcinoma.All of eight cases received chemotherapy following operation.No serious surgical wound infection or abdominal infection occurred after the first cycle of chemotherapy.The other three cases were diagnosed by endoscopic pathology,and treated with chemotherapy.One of them received autologous hematopoietic stem cell transplantation,and one of them underwent intestinal resection and anastomosis because of intestinal obstruction during chemotherapy.Results Ten patients died,with a median overall survival of 14.5 months(range,3-25 months).One patient was alive with a follow-up for 70 months.Conclusions MEITL is a rare lymphoma with insidious onset.Acute abdomen is often the first symptom.The main treatment of MEITL is chemotherapy,while surgery also has a role in the treatment of acute complications and the access of pathological specimen.