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Gastrointestinal lymphoma 

Gastrointestinal lymphoma

Chapter:
Gastrointestinal lymphoma
Author(s):

P.G. Isaacson

DOI:
10.1093/med/9780199204854.003.151004

A relevant case history from Oxford Case Histories in Gastroenterology and Hepatology has been added to this chapter.

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date: 30 March 2017

Primary gastrointestinal lymphoma, which is the commonest extranodal lymphoma and almost exclusively of non-Hodgkin’s type, is defined as lymphoma that has presented with the main bulk of disease in the gastrointestinal tract, with or without involvement of contiguous lymph nodes, and necessitating direction of treatment to that site.

MALT lymphoma describes a group of low-grade B-cell lymphomas whose histology recapitulates the features of mucosa-associated lymphoid tissue (MALT). It most commonly affects the stomach, presenting with nonspecific dyspepsia. Endoscopy typically shows inflamed or eroded mucosa rather than tumour mass. Many if not all cases appear to be driven by Helicobacter pylori, with 75% regressing following eradication of the organism with appropriate antibiotics. Deeply invasive lymphomas and those with adverse histological or cytogenetic features are unlikely to respond.

Enteropathy-associated T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T lymphocytes that occurs most commonly in the jejunum or ileum and is sometimes associated with coeliac disease. It presents with abdominal pain, often due to intestinal perforation, and in some cases there is a prodromal period of refractory coeliac disease (sometimes accompanied by ulcerative jejunitis). The prognosis is usually poor, with death frequently resulting from abdominal complications in patients already weakened by uncontrolled malabsorption.

Burkitt’s lymphoma is the most frequent childhood gastrointestinal lymphoma and is particularly common in the Middle East. B-cell lymphoproliferative conditions associated with immunodeficiency commonly present in the gastrointestinal tract and are increasingly important.

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