摘要: |
目的 总结单形性亲上皮肠道T细胞淋巴瘤(monomorphic epitheliotropic intestinal T-cell lymphoma,MEITL)的临床病理学特征及预后。方法 回顾性分析复旦大学附属中山医院2014年1月至2021年3月诊治的MEITL患者的临床资料。结果 共纳入7例患者,均为男性,诊断中位年龄为55(33~77)岁,原发灶最常见于小肠。腹痛、腹泻及体质量下降是主要临床症状,肠穿孔为主要并发症。6例患者通过外科手术确诊,另1例患者通过胃镜活检病理确诊。手术切除患者中,4例患者因小肠穿孔或出血行急诊手术。CD8、T细胞胞内抗原1及CD56在肿瘤组织中高表达,所有患者均不表达EB病毒编码RNA。5例患者诊断分期晚,2例患者诊断分期相对早。5例患者接受了化疗,其中3例患者接受了西达本胺联合化疗方案。随访期间5例患者死亡,2例接受择期手术的早期患者病情稳定;随访截至2021年11月9日,患者总生存期为4 d~12个月。结论 诊断晚期及严重并发症可能导致MEITL患者预后差,接受择期手术、化疗应答好患者预后相对好,亟需新的诊断及治疗方法改善MEITL预后。 |
关键词: 临床病理特征 胃肠道 单形性亲上皮肠道T细胞淋巴瘤 |
DOI:10.12025/j.issn.1008-6358.2022.20212967 |
分类号: |
基金项目:国家自然科学基金(81502596,81970505). |
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Monomorphic epitheliotropic intestinal T-cell lymphoma: a retrospective analysis in single center |
ZHANG Guang-cong1, YIN Jie1, CHEN Ling-li2, LIU Tao-tao1, DONG Ling1, SHEN Xi-zhong1,3
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1.Department of Gastroenterology, Zhongshan Hospital, Fudan University, Shanghai 200032, China;2.Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai 200032, China;3.Shanghai Institute of Liver Diseases, Shanghai 200032, China
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Abstract: |
Objective To summarize the clinicopathologic characteristics and outcomes of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). Methods The clinical profiles of patients diagnosed with MEITL in Zhongshan Hospital, Fudan University from Jan. 2014 to Mar. 2021 were retrospective analyzed. Results All 7 patients were male. The median age of them at diagnosis was 55 (33-77) years old. The most frequent site of lesion involved was small intestine. Symptoms including abdominal pain, diarrhea, and weigh loss were common, and intestinal perforation occurred in most patients. Six patients were diagnosed through surgical resection of lesions, another patient was confirmed pathologically by gastroscope biopsy. Among patients underwent surgical resection, emergency surgeries were carried out in four patients to manage intestinal perforation or bleeding. Most tumors expressed CD8, T-cell intracellular antigen-1, and CD56, while none expressed Epstein-Barr virus-encoded RNA. Five patients were diagnosed at late stage, while another two were at relatively early stage. Five patients received chemotherapy, and chidamide-based combination regimens were used in 3 patients. Five patients died and another 2 patients at early stage who undergone selective surgery survived. Up to November 9, 2021, overall survival of this cohort was less than one year. Conclusions Late stage at diagnosis and severe complications may contribute to dismal prognosis, while the prognosis of patients with selective surgery, good response to chemotherapy may be relatively improved. Novel diagnostic and therapeutic approaches are required to improve survival of MEITL patients. |
Key words: clinicopathologic characteristic gastrointestinal tract monomorphic epitheliotropic intestinal T-cell lymphoma |