单形性亲上皮性肠道T细胞淋巴瘤：附3例患者临床病理分析_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 杨迷玲 ¹ , 王立峰 ² , 徐小艳 ¹ , 姜黄 ¹

1. ;
2. ;

Corresponding author：

杨迷玲, Email: yml0922@163.com

Keywords：

单形性亲上皮性肠道T细胞淋巴瘤; 临床病理; 免疫组织化学

DOI：

10.7507/1007-9424.202404086

Video：

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目的总结单形性亲上皮性肠道T细胞淋巴瘤（monomorphic epitheliotropic intestinal T-cell lymphoma，MEITL）的临床病理特征。方法回顾性收集2014年4月至2023年4月期间郑州人民医院收治的3例MEITL患者的临床病理资料。结果 3例患者中，1例为女性、2例为男性；3例患者病变均位于小肠；临床表现以腹痛为主，2例患者出现肠梗阻、肠穿孔，1例患者伴出汗、发冷等症状且伴轻度贫血。3例患者均行手术治疗，术后均接受化疗，2例行骨髓学检查均未见侵犯；术后随访，2例死亡，1例存活。光镜下组织病理学特点：肿瘤细胞弥漫浸润肠壁全层，肿瘤细胞形态单一，小至中等大小，核圆形，染色质致密，核仁不明显；黏膜表面可见溃疡，小肠绒毛结构破坏，可见“亲上皮现象”、肿瘤性坏死及较多核分裂象。免疫组织化学染色结果：CD3、CD7、CD8、CD56及T细胞胞浆内抗原均呈阳性表达，颗粒酶B和穿孔素部分阳性表达，CD20、CD79α、CD5、Pax-5、CD30及末端脱氧核苷酸转移酶均阴性表达，Ki67增殖指数约70%~90%。原位杂交检测Epstein-Barr病毒编码的小RNA均阴性。结论 MEITL是肠道罕见的高度侵袭性的T细胞淋巴瘤，大多发生于小肠，临床表现无特异性，具有独特的组织病理学特征，明确诊断需结合组织病理学、免疫组织化学以及分子检测结果。

Citation： 杨迷玲, 王立峰, 徐小艳, 姜黄. 单形性亲上皮性肠道T细胞淋巴瘤：附3例患者临床病理分析. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2024, 31(9): 1125-1129. doi: 10.7507/1007-9424.202404086 Copy

1.	Campo E, Swerdlow SH, Harris NL, et al. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood, 2011, 117(19): 5019-5032.
2.	Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 2016, 127(20): 2375-2390.
3.	Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia, 2022, 36(7): 1720-1748.
4.	王晓敏, 付刚, 陈红芳, 等. 原发于胃的单形性嗜上皮性T细胞淋巴瘤1例. 中国肿瘤临床, 2020, 47(1): 54.
5.	Zhong H, Zheng Y, Zhang F. Monomorphic epitheliotropic intestinal T-cell lymphomas: a case report. Diagn Pathol, 2021, 16(1): 80. doi: 10.1186/s13000-021-01143-x.
6.	张旭, 董菲, 田磊, 等. 单形性嗜上皮性肠道T细胞淋巴瘤11例临床分析. 中国微创外科杂志, 2022, 22(3): 232-236.
7.	罗瑜, 邱逦. 卵巢单形性亲上皮性肠道T细胞淋巴瘤1例. 中国医学影像技术, 2022, 38(5): 796.
8.	Suzuki Y, Minemura H, Tomita H, et al. Monomorphic epitheliotropic intestinal T-cell lymphoma involving the lung and brain: a rare case study. Intern Med, 2020, 59(20): 2559-2563.
9.	Yanagi M, Komura T, Kagaya T. A rare cause of jejunal perforation: Monomorphic epitheliotropic intestinal T-cell lymphoma. JGH Open, 2022, 6(12): 915-917.
10.	胡泽玉, 周铖, 杨林, 等. 单形性嗜上皮性肠T细胞淋巴瘤致反复消化道出血1例. 上海交通大学学报(医学版), 2023, 43(1): 132-136.
11.	Silva JF, Medas R, Fonseca E, et al. A rare case of monomorphic epitheliotropic intestinal T-cell lymphoma mimicking Crohn’s disease. Rev Esp Enferm Dig, 2022, 114(8): 497-498.
12.	陈春妮, 王震, 江悦, 等. 肠道单形性亲上皮性T细胞淋巴瘤12例临床病理学特征. 中华病理学杂志, 2020, 49(1): 17-21.
13.	张炼, 喻芳, 韦菊英, 等. 单形性嗜上皮肠道T细胞淋巴瘤的临床特征分析. 浙江医学, 2023, 45(5): 487-492, 503.
14.	袁风菊, 丁兀兀, 李慧, 等. 异常表达CD79α的单形性嗜上皮肠道T细胞淋巴瘤1例. 诊断病理学杂志, 2022, 29(9): 881-882.
15.	Alves de Castro JV, Costa FD, Ferreira CR. Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract—a report of two cases of a new provisional entity on the World Health Organization classification of hematolymphoid tumors. Int J Surg Pathol, 2023, 31(5): 596-599.
16.	Liu TZ, Zheng YJ, Zhang ZW, et al. Chidamide based combination regimen for treatment of monomorphic epitheliotropic intestinal T cell lymphoma following radical operation: Two case reports. World J Clin Cases, 2020, 8(7): 1278-1286.
17.	Yi JH, Lee GW, Do YR, et al. Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. Ann Hematol, 2019, 98(11): 2541-2550.
18.	Ozaka S, Inoue K, Okajima T, et al. Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature. World J Gastroenterol, 2021, 27(38): 6501-6510.

1. Campo E, Swerdlow SH, Harris NL, et al. The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications. Blood, 2011, 117(19): 5019-5032.
2. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 2016, 127(20): 2375-2390.
3. Alaggio R, Amador C, Anagnostopoulos I, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia, 2022, 36(7): 1720-1748.
4. 王晓敏, 付刚, 陈红芳, 等. 原发于胃的单形性嗜上皮性T细胞淋巴瘤1例. 中国肿瘤临床, 2020, 47(1): 54.
5. Zhong H, Zheng Y, Zhang F. Monomorphic epitheliotropic intestinal T-cell lymphomas: a case report. Diagn Pathol, 2021, 16(1): 80. doi: 10.1186/s13000-021-01143-x.
6. 张旭, 董菲, 田磊, 等. 单形性嗜上皮性肠道T细胞淋巴瘤11例临床分析. 中国微创外科杂志, 2022, 22(3): 232-236.
7. 罗瑜, 邱逦. 卵巢单形性亲上皮性肠道T细胞淋巴瘤1例. 中国医学影像技术, 2022, 38(5): 796.
8. Suzuki Y, Minemura H, Tomita H, et al. Monomorphic epitheliotropic intestinal T-cell lymphoma involving the lung and brain: a rare case study. Intern Med, 2020, 59(20): 2559-2563.
9. Yanagi M, Komura T, Kagaya T. A rare cause of jejunal perforation: Monomorphic epitheliotropic intestinal T-cell lymphoma. JGH Open, 2022, 6(12): 915-917.
10. 胡泽玉, 周铖, 杨林, 等. 单形性嗜上皮性肠T细胞淋巴瘤致反复消化道出血1例. 上海交通大学学报(医学版), 2023, 43(1): 132-136.
11. Silva JF, Medas R, Fonseca E, et al. A rare case of monomorphic epitheliotropic intestinal T-cell lymphoma mimicking Crohn’s disease. Rev Esp Enferm Dig, 2022, 114(8): 497-498.
12. 陈春妮, 王震, 江悦, 等. 肠道单形性亲上皮性T细胞淋巴瘤12例临床病理学特征. 中华病理学杂志, 2020, 49(1): 17-21.
13. 张炼, 喻芳, 韦菊英, 等. 单形性嗜上皮肠道T细胞淋巴瘤的临床特征分析. 浙江医学, 2023, 45(5): 487-492, 503.
14. 袁风菊, 丁兀兀, 李慧, 等. 异常表达CD79α的单形性嗜上皮肠道T细胞淋巴瘤1例. 诊断病理学杂志, 2022, 29(9): 881-882.
15. Alves de Castro JV, Costa FD, Ferreira CR. Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract—a report of two cases of a new provisional entity on the World Health Organization classification of hematolymphoid tumors. Int J Surg Pathol, 2023, 31(5): 596-599.
16. Liu TZ, Zheng YJ, Zhang ZW, et al. Chidamide based combination regimen for treatment of monomorphic epitheliotropic intestinal T cell lymphoma following radical operation: Two case reports. World J Clin Cases, 2020, 8(7): 1278-1286.
17. Yi JH, Lee GW, Do YR, et al. Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. Ann Hematol, 2019, 98(11): 2541-2550.
18. Ozaka S, Inoue K, Okajima T, et al. Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature. World J Gastroenterol, 2021, 27(38): 6501-6510.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

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单形性亲上皮性肠道T细胞淋巴瘤：附3例患者临床病理分析