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胃肠道CD8阳性惰性T细胞淋巴增殖性疾病:一例报告并文献复习

CD8-positive indolent T-Cell lymphoproliferative disorder of the gastrointestinal tract: A case report and review of literature.

作者信息

Weng Chun-Yan, Ye Cheng, Fan Yi-Hong, Lv Bin, Zhang Chun-Li, Li Meng

机构信息

Department of Gastroenterology, The First Clinical Medical College of Zhejiang Chinese Medical University, Hangzhou 310053, Zhejiang Province, China.

Department of Gastroenterology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China.

出版信息

World J Clin Cases. 2022 May 26;10(15):4971-4984. doi: 10.12998/wjcc.v10.i15.4971.

Abstract

BACKGROUND

Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (ITLPD-GI), a primary tumor forming in the gastrointestinal (GI) tract, represents a rarely diagnosed clonal T-cell disease with a protracted clinical course.

CASE SUMMARY

This report presented a 45-year-old male patient with a 6-year history of anal fistula and a more than 10-year history of recurrent diarrhea who was not correctly diagnosed until the occurrence of complications such as intestinal perforation. Postsurgical histopathological analysis, combined with hematoxylin-eosin staining, immunohistochemistry and TCRβ/γ clonal gene rearrangement test, confirmed the diagnosis of CD8+ ITLPD-GI.

CONCLUSION

Individuals with this scarce lymphoma frequently show non-specific symptoms that are hard to recognize. So far, indolent CD8+ ITLPD-GI has not been comprehensively examined. The current mini-review focused on evaluating indolent CD8+ ITLPD-GI cases based on existing literature and discussing future directions for improved differential diagnosis, detection of genetic and epigenetic alterations, and therapeutic target identification.

摘要

背景

胃肠道惰性T细胞淋巴增殖性疾病(ITLPD-GI)是一种在胃肠道形成的原发性肿瘤,是一种临床病程迁延、诊断罕见的克隆性T细胞疾病。

病例摘要

本报告介绍了一名45岁男性患者,有6年肛瘘病史和10多年反复腹泻病史,直到发生肠穿孔等并发症才得以正确诊断。术后组织病理学分析,结合苏木精-伊红染色、免疫组织化学和TCRβ/γ克隆基因重排检测,确诊为CD8+ ITLPD-GI。

结论

这种罕见淋巴瘤患者常表现出难以识别的非特异性症状。迄今为止,惰性CD8+ ITLPD-GI尚未得到全面研究。本综述聚焦于基于现有文献评估惰性CD8+ ITLPD-GI病例,并讨论改善鉴别诊断、检测基因和表观遗传改变以及确定治疗靶点的未来方向。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d151/9198890/94079df0ec58/WJCC-10-4971-g001.jpg

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