A case of primary intestinal lymphangiectasia with non-Hodgkin lymphoma

Doudou Hu,Xiangjun Jiang,Xin Guan,Jian Zhang,Wanlei Ren,Xianghua Cui

doi:10.1186/s12876-021-01997-x

Doudou Hu, Xiangjun Jiang + Show 4 more

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https://doi.org/10.1186/s12876-021-01997-x

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Abstract

BackgroundPrimary 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 presentationA 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.ConclusionOur report supports the hypothesis that PIL is associated with lymphoma development.

Highlights

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen
Our report supports the hypothesis that PIL is associated with lymphoma development
Primary intestinal lymphangiectasia (PIL) is a rare disease manifested by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen [1], which in turn leads to the suppression of both the humoral and cellular immune systems [2]

Summary

Introduction

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing enteropathy characterized by the loss of proteins, lymphocytes, and immunoglobulins into the intestinal lumen. Conclusion: Our report supports the hypothesis that PIL is associated with lymphoma development. We report a case of diffuse large B-cell lymphoma occurring 23 years after the onset of PIL, and *Correspondence: drjxj11@sohu.com 1 Department of Gastroenterology, Qingdao Municipal Hospital, Jiaozhou Road 1#, Qingdao 266071, People’s Republic of China Full list of author information is available at the end of the article conduct a systematic review of the literature on lymphoma and PIL.

Results

Conclusion