JLE

Hématologie

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T-cell lymphoma and celiac disease Volume 5, issue 2, Mars - Avril 1999

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Primitive gastro-intestinal lymphomas are the most frequent localization of primitive extra-nodal lymphoma. T-cell gastro-intestinal lym-phomas are rare and heterogeneous. T-cell intestinal lymphomas associated with an enteropathy (EATCL) are the best characterized. They are large cell lymphomas with a poor prognosis. They are characterized by their CD3+, CD4–, CD8–, CD103+, TIA1+, granzyme B+ phenotype and by their epitheliotropism. The phenotype and the epitheliotropism have suggested the possible origin from intestinal intraepithelial lymphocytes. Recent cases of purely intraepithelial lymphomas have been described, particularly associated with clinical and biological manifestations of refractory sprue. The raise of a lymphoma, invasive or intraepithelial, as a complication of adult celiac disease seems favoured by a dietary non compliance or inadvertent gluten intake. The histological diagnosis of lymphoma is very difficult to assess. However diagnosis is helped by phenotypic and molecular studies of intestinal biopsies. Rare other forms of intestinal T cell lymphoma have been described, including lymphoma with eosinophilia, ulcerative jejunitis and small intestinal T-cell lymphoproliferation.