Enteropathy-associated T-cell lymphoma

Enteropathy-Associated T-cell Lymphoma (EATL) is a T-cell lymphoma of the small intestine. It is the most common primary gastrointestinal T-cell lymphoma, arising from the T cells that are found between the cells that line the small intestinal (brush border cells or small intestinal epithelial cells).&lt;ref&gt;Isaacson, PG. Intestinal Lymphoma (1994) 25(10):1020-9&lt;/ref&gt; These cancerous T-cells are a consequence of prolonged, untreated coeliac disease in genetically susceptible individuals.

Genetics
Enteropathy associated T-cell lymphoma (EATL) is environmentally induced as a result of the consumption of Triticeae glutens (e.g. wheat gluten). In gluten-sensitive individuals with EATL, 68% are homozygotes of the DQB1 subtype at the HLA-DQB1 locus .&lt;ref name="pmid17470479"&gt;&lt;/ref&gt; (See Coeliac Disease, HLA-DQ, HLA DR3-DQ2) A DQ isoform that appears to be responsible for EATL in the overwhelming number of cases is highly effective at presenting a proteolytically protected region of α2-glaidin to T-cells, constant over-stimulation of T-cell eventually results in neoplastic growth.&lt;ref name="pmid17190762"&gt;&lt;/ref&gt; EATL typically appears after the 4th decade of life, within 3 years of coeliac disease diagnosis or in undiagnosed coeliacs.&lt;ref name="pmid16979946"&gt;&lt;/ref&gt;&lt;ref name="pmid16527694"&gt;&lt;/ref&gt; In treated coeliacs, EATL may be preceded by refractory coeliac disease 1(RCD1) or, prominently, refractory celiac disease 2 (RCD2), in which EATL is a frequent outcome&lt;ref name="pmid17410290"&gt;&lt;/ref&gt; Refractory coeliac disease is no longer favorably responsive to wheat-gluten abstinence. Beyond the RCD1 stage, many drugs are not effective, and undetected coeliac disease leading to de novo EATL generally has a poor outcome.

Early recognition of coeliac disease, particularly with a focus on DQ2 homozygotes and in affected family members, is the only effective prevention, though bone marrow transplant was suggested as a treatment during early RCD2.&lt;ref name="pmid15696854"&gt;&lt;/ref&gt;