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原发性小肠结外 NK/T 细胞淋巴瘤,鼻型伴肾脏累及:一例罕见病例报告及文献复习。

Primary small intestinal extranodal NK/T cell lymphoma, nasal type with kidney involvement: a rare case report and literature review.

机构信息

Department of Pathology, Shanghai Pudong New Area People's Hospital, No.490, Chuanhuan South Road, Chuansha town, 200120, Shanghai, China.

Department of Pathology, Xuzhou Central Hospital, No.199, Jiefang South Road, 221009, Xuzhou, Jiangsu, China.

出版信息

Diagn Pathol. 2022 Oct 5;17(1):75. doi: 10.1186/s13000-022-01254-z.

DOI:10.1186/s13000-022-01254-z
PMID:36199094
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9533626/
Abstract

BACKGROUND

Extranodal NK/T cell lymphoma, nasal type (EN-NK/T-NT) is a rare and aggressive type of non-Hodgkin's lymphoma. EN-NK/T-NT seldom occurs in the gastrointestinal tract, and renal involvement is relatively rare.

CASE PRESENTATION

Here we report a case of primary small intestinal EN-NK/T-NT with kidney involvement. We present the case of a 71-year-old female who was admitted to our hospital for coronary heart disease with a fever of unknown origin. Laboratory examination showed renal impairment and PET/CT showed a locally thickened wall of the small intestine, abnormally increased FDG metabolism in the right lower abdomen, and multiple slightly high-density masses with abnormal increased FDG metabolism in the right kidney. The gross specimen showed a grayish-white lump located in the ileum approximately 15 cm away from the ileocecum, and two grayish-white lumps located in the upper and lower poles of the right kidney, respectively. The pathological diagnosis was EN-NK/T-NT. The patient died approximately 10 months after the operation.

CONCLUSION

EN-NK/T-NT is a rare type of non-Hodgkin's lymphoma and may develop insidiously, with fever as the only clinical manifestation. The disease was found to be difficult to diagnose in the early stage, resulting in a highly aggressive clinical course and short survival time.

摘要

背景

结外 NK/T 细胞淋巴瘤,鼻型(EN-NK/T-NT)是一种罕见且侵袭性很强的非霍奇金淋巴瘤。EN-NK/T-NT 在胃肠道中很少见,肾脏受累也相对少见。

病例介绍

本文报告了一例原发于小肠的伴有肾脏受累的 EN-NK/T-NT。我们报告了一例 71 岁女性患者,因冠心病发热原因不明而入院。实验室检查显示肾功能损害,PET/CT 显示小肠局部壁增厚,右下腹部 FDG 代谢异常增高,右肾有多个稍高密度肿块,FDG 代谢异常增高。大体标本显示距回盲部约 15cm 的回肠处有一个灰白色肿块,右肾上、下极各有一个灰白色肿块。病理诊断为 EN-NK/T-NT。术后约 10 个月患者死亡。

结论

EN-NK/T-NT 是一种罕见的非霍奇金淋巴瘤,可能隐匿起病,以发热为唯一临床表现。该病早期诊断困难,导致侵袭性临床病程和短生存期。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/fbcc79bc20db/13000_2022_1254_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/c0571c081629/13000_2022_1254_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/cdea3ba0bf39/13000_2022_1254_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/fbcc79bc20db/13000_2022_1254_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/c0571c081629/13000_2022_1254_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/cdea3ba0bf39/13000_2022_1254_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5e7/9533626/fbcc79bc20db/13000_2022_1254_Fig3_HTML.jpg

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