單形性親上皮性腸道T細胞淋巴瘤：附3例患者臨床病理分析_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 楊迷玲 ¹ , 王立峰 ² , 徐小艷 ¹ , 姜黃 ¹

1. ;
2. ;

Corresponding?author：

楊迷玲, Email: yml0922@163.com

Keywords：

單形性親上皮性腸道T細胞淋巴瘤; 臨床病理; 免疫組織化學

DOI：

10.7507/1007-9424.202404086

Video：

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目的總結單形性親上皮性腸道T細胞淋巴瘤（monomorphic epitheliotropic intestinal T-cell lymphoma，MEITL）的臨床病理特征。方法回顧性收集2014年4月至2023年4月期間鄭州人民醫院收治的3例MEITL患者的臨床病理資料。結果 3例患者中，1例為女性、2例為男性；3例患者病變均位于小腸；臨床表現以腹痛為主，2例患者出現腸梗阻、腸穿孔，1例患者伴出汗、發冷等癥狀且伴輕度貧血。3例患者均行手術治療，術后均接受化療，2例行骨髓學檢查均未見侵犯；術后隨訪，2例死亡，1例存活。光鏡下組織病理學特點：腫瘤細胞彌漫浸潤腸壁全層，腫瘤細胞形態單一，小至中等大小，核圓形，染色質致密，核仁不明顯；黏膜表面可見潰瘍，小腸絨毛結構破壞，可見“親上皮現象”、腫瘤性壞死及較多核分裂象。免疫組織化學染色結果：CD3、CD7、CD8、CD56及T細胞胞漿內抗原均呈陽性表達，顆粒酶B和穿孔素部分陽性表達，CD20、CD79α、CD5、Pax-5、CD30及末端脫氧核苷酸轉移酶均陰性表達，Ki67增殖指數約70%~90%。原位雜交檢測Epstein-Barr病毒編碼的小RNA均陰性。結論 MEITL是腸道罕見的高度侵襲性的T細胞淋巴瘤，大多發生于小腸，臨床表現無特異性，具有獨特的組織病理學特征，明確診斷需結合組織病理學、免疫組織化學以及分子檢測結果。

Citation： 楊迷玲, 王立峰, 徐小艷, 姜黃. 單形性親上皮性腸道T細胞淋巴瘤：附3例患者臨床病理分析. 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.	王曉敏, 付剛, 陳紅芳, 等. 原發于胃的單形性嗜上皮性T細胞淋巴瘤1例. 中國腫瘤臨床, 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.	張旭, 董菲, 田磊, 等. 單形性嗜上皮性腸道T細胞淋巴瘤11例臨床分析. 中國微創外科雜志, 2022, 22(3): 232-236.
7.	羅瑜, 邱邐. 卵巢單形性親上皮性腸道T細胞淋巴瘤1例. 中國醫學影像技術, 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.	胡澤玉, 周鋮, 楊林, 等. 單形性嗜上皮性腸T細胞淋巴瘤致反復消化道出血1例. 上海交通大學學報(醫學版), 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.	陳春妮, 王震, 江悅, 等. 腸道單形性親上皮性T細胞淋巴瘤12例臨床病理學特征. 中華病理學雜志, 2020, 49(1): 17-21.
13.	張煉, 喻芳, 韋菊英, 等. 單形性嗜上皮腸道T細胞淋巴瘤的臨床特征分析. 浙江醫學, 2023, 45(5): 487-492, 503.
14.	袁風菊, 丁兀兀, 李慧, 等. 異常表達CD79α的單形性嗜上皮腸道T細胞淋巴瘤1例. 診斷病理學雜志, 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. 王曉敏, 付剛, 陳紅芳, 等. 原發于胃的單形性嗜上皮性T細胞淋巴瘤1例. 中國腫瘤臨床, 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. 張旭, 董菲, 田磊, 等. 單形性嗜上皮性腸道T細胞淋巴瘤11例臨床分析. 中國微創外科雜志, 2022, 22(3): 232-236.
7. 羅瑜, 邱邐. 卵巢單形性親上皮性腸道T細胞淋巴瘤1例. 中國醫學影像技術, 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. 胡澤玉, 周鋮, 楊林, 等. 單形性嗜上皮性腸T細胞淋巴瘤致反復消化道出血1例. 上海交通大學學報(醫學版), 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. 陳春妮, 王震, 江悅, 等. 腸道單形性親上皮性T細胞淋巴瘤12例臨床病理學特征. 中華病理學雜志, 2020, 49(1): 17-21.
13. 張煉, 喻芳, 韋菊英, 等. 單形性嗜上皮腸道T細胞淋巴瘤的臨床特征分析. 浙江醫學, 2023, 45(5): 487-492, 503.
14. 袁風菊, 丁兀兀, 李慧, 等. 異常表達CD79α的單形性嗜上皮腸道T細胞淋巴瘤1例. 診斷病理學雜志, 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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單形性親上皮性腸道T細胞淋巴瘤：附3例患者臨床病理分析