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表现为小肠显著绒毛萎缩的单形上皮嗜肠性T细胞淋巴瘤

Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Presenting With Significant Villous Atrophy in the Small Intestine.

作者信息

Ozaka Sotaro, Takahashi Haruhiko, Hamano Tomoe, Fukuda Masahide, Mizukami Kazuhiro

机构信息

Department of Gastroenterology, Faculty of Medicine, Oita University, Yufu, JPN.

出版信息

Cureus. 2025 Feb 23;17(2):e79496. doi: 10.7759/cureus.79496. eCollection 2025 Feb.

DOI:10.7759/cureus.79496
PMID:40134998
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11936312/
Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare and aggressive primary intestinal T-cell lymphoma with a high mortality rate and poor prognosis. Although endoscopy plays a key role in early diagnosis, reports on detailed endoscopic findings are limited. Here, we present a case of MEITL with refractory diarrhea and significant villous atrophy, as observed on endoscopy. A 67-year-old woman was admitted to our hospital with refractory diarrhea and weight loss. Esophagogastroduodenoscopy revealed microgranular mucosa with significant villous atrophy in the duodenum. Enhanced magnifying endoscopy with narrow band imaging showed flattening and loss pattern of the villi in the transverse part of the duodenum. Colonoscopy also showed significant villous atrophy in the ileum. Biopsy specimens from the duodenum and ileum showed diffuse proliferation of small- to medium-sized atypical lymphoid cells in the lamina propria and intraepithelial lymphocytes. Immunohistochemistry revealed that the cells were positive for CD3, CD8, CD56, and Granzyme B. Diagnosing MEITL, PVPP (sobuzoxane, etoposide, and prednisone) chemotherapy was administered. However, since the patient developed intestinal obstruction after two courses of chemotherapy, it was discontinued. The patient died of intestinal perforation 82 days after diagnosis. MEITL can cause villous atrophy in the small intestine. Hence, magnifying endoscopy and follow-up histological examination are essential when villous atrophy is observed in patients with refractory diarrhea.

摘要

单形性上皮趋化性肠道T细胞淋巴瘤(MEITL)是一种罕见且侵袭性强的原发性肠道T细胞淋巴瘤,死亡率高,预后差。尽管内镜检查在早期诊断中起关键作用,但关于详细内镜检查结果的报道有限。在此,我们报告一例MEITL病例,内镜检查发现有难治性腹泻和明显的绒毛萎缩。一名67岁女性因难治性腹泻和体重减轻入院。食管胃十二指肠镜检查显示十二指肠黏膜呈微颗粒状,伴有明显的绒毛萎缩。窄带成像放大内镜检查显示十二指肠横部绒毛呈扁平及缺失形态。结肠镜检查也显示回肠有明显的绒毛萎缩。十二指肠和回肠活检标本显示固有层和上皮内淋巴细胞中小至中等大小的非典型淋巴样细胞弥漫性增生。免疫组织化学显示这些细胞CD3、CD8、CD56和颗粒酶B呈阳性。诊断为MEITL后,给予PVPP(司莫司汀、依托泊苷和泼尼松)化疗。然而,由于患者在两个疗程化疗后出现肠梗阻,化疗停止。患者在诊断后82天死于肠穿孔。MEITL可导致小肠绒毛萎缩。因此,对于难治性腹泻患者,当观察到绒毛萎缩时,放大内镜检查及后续组织学检查至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/1a13bda4a20d/cureus-0017-00000079496-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/01cd45c189af/cureus-0017-00000079496-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/6984196df088/cureus-0017-00000079496-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/36adb132e475/cureus-0017-00000079496-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/1a13bda4a20d/cureus-0017-00000079496-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/01cd45c189af/cureus-0017-00000079496-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/6984196df088/cureus-0017-00000079496-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/36adb132e475/cureus-0017-00000079496-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bca5/11936312/1a13bda4a20d/cureus-0017-00000079496-i04.jpg

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本文引用的文献

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ACG Case Rep J. 2024 Nov 20;11(11):e01549. doi: 10.14309/crj.0000000000001549. eCollection 2024 Nov.
2
Narrow-band imaging for optical diagnosis of duodenal villous atrophy in patients with suspected coeliac disease: A systematic review and meta-analysis.窄带成像在疑似乳糜泻患者的十二指肠绒毛萎缩光学诊断中的应用:系统评价和荟萃分析。
Dig Liver Dis. 2024 Jun;56(6):971-977. doi: 10.1016/j.dld.2023.08.053. Epub 2023 Sep 3.
3
Successful early diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma manifesting as chronic diarrhea and hypokalemia using video capsule endoscopy and double-balloon enteroscopy.
应用视频胶囊内镜和双气囊小肠镜成功诊断表现为慢性腹泻和低钾血症的单相上皮样肠道 T 细胞淋巴瘤。
Clin J Gastroenterol. 2022 Oct;15(5):913-919. doi: 10.1007/s12328-022-01676-8. Epub 2022 Jul 30.
4
Initial and advanced endoscopic findings of monomorphic epitheliotropic intestinal T-cell lymphoma in the duodenum: A case report.十二指肠单形性上皮样肠道T细胞淋巴瘤的初始及高级内镜检查结果:一例报告
DEN Open. 2022 Apr 1;2(1):e118. doi: 10.1002/deo2.118. eCollection 2022 Apr.
5
Monomorphic epitheliotropic intestinal T-cell lymphoma comprises morphologic and genomic heterogeneity impacting outcome.单一表型上皮样肠道 T 细胞淋巴瘤具有影响预后的形态学和基因组异质性。
Haematologica. 2023 Jan 1;108(1):181-195. doi: 10.3324/haematol.2022.281226.
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