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原发性肠道淋巴管扩张症合并非霍奇金淋巴瘤 1 例

A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma.

机构信息

Department of Gastroenterology, Qingdao Municipal Hospital, Jiaozhou Road 1#, Qingdao, 266071, People's Republic of China.

Department of Traditional Chinese Medicine, Qingdao Central Hospital, Qingdao, 266042, People's Republic of China.

出版信息

BMC Gastroenterol. 2021 Dec 11;21(1):461. doi: 10.1186/s12876-021-01997-x.

DOI:10.1186/s12876-021-01997-x
PMID:34895151
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8665534/
Abstract

BACKGROUND

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Increasing evidence has demonstrated an association between PIL and lymphoma.

CASE PRESENTATION

A 54-year-old man with a 20-year history of abdominal distension and bilateral lower limb edema was admitted. Laboratory investigations revealed lymphopenia, hypoalbuminemia, decreased triglyceride and cholesterol level. Colonoscopy showed multiple smooth pseudo polyps in the ileocecal valve and terminal ileum and histological examination showed conspicuous dilation of the lymphatic channels in the mucosa and submucosa. A diagnosis of PIL was made. Three years later colonoscopy of the patient showed an intraluminal proliferative mass in the ascending colon and biopsy examination confirmed a malignant non-Hodgkin lymphoma. Then the patient was been underwent chemotherapy, and his clinical condition is satisfactory.

CONCLUSION

Our report supports the hypothesis that PIL is associated with lymphoma development.

摘要

背景

原发性肠淋巴管扩张症(PIL)是一种罕见的蛋白丢失性肠病,其特征是蛋白质、淋巴细胞和免疫球蛋白丢失到肠腔中。越来越多的证据表明 PIL 与淋巴瘤之间存在关联。

病例介绍

一名 54 岁男性,有 20 年腹胀和双下肢水肿病史,入院。实验室检查显示淋巴细胞减少、低白蛋白血症、甘油三酯和胆固醇水平降低。结肠镜检查显示回盲瓣和末端回肠有多发性光滑假息肉,组织学检查显示黏膜和黏膜下层淋巴管明显扩张。诊断为 PIL。三年后,患者的结肠镜检查显示升结肠腔内有一个增殖性肿块,活检证实为恶性非霍奇金淋巴瘤。随后患者接受了化疗,其临床状况良好。

结论

我们的报告支持 PIL 与淋巴瘤发展相关的假说。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/00f6d951217e/12876_2021_1997_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/b67f48a9c0e4/12876_2021_1997_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/f3ce776a064d/12876_2021_1997_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/00f6d951217e/12876_2021_1997_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/b67f48a9c0e4/12876_2021_1997_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/f3ce776a064d/12876_2021_1997_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2d1/8665534/00f6d951217e/12876_2021_1997_Fig3_HTML.jpg

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Dig Dis. 2019;37(6):458-466. doi: 10.1159/000499450. Epub 2019 Apr 26.
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Intestinal Lymphangiectasia: Insights on Management and Literature Review.肠淋巴管扩张症:管理见解与文献综述
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