Enteropathy-associated T-cell lymphoma

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Template:Infobox Disease 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).<ref>Isaacson, PG. Intestinal Lymphoma (1994) 25(10):1020-9</ref> 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*02 subtype at the HLA-DQB1 locus .<ref name="pmid17470479">Al-Toma A, Verbeek WH, Hadithi M, von Blomberg BM, Mulder CJ (2007). [Expression error: Missing operand for > "Survival in Refractory Coeliac Disease and Enteropathy associated T cell Lymphoma: Retrospective evaluation of single centre experience"]. Gut 56 (10): 1373. doi:10.1136/gut.2006.114512. PMID 17470479. </ref> (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.<ref name="pmid17190762">Jores RD, Frau F, Cucca F, et al. (2007). [Expression error: Missing operand for > "HLA-DQB1*0201 homozygosis predisposes to severe intestinal damage in celiac disease"]. Scand. J. Gastroenterol. 42 (1): 48–53. doi:10.1080/00365520600789859. PMID 17190762. </ref> EATL typically appears after the 4th decade of life, within 3 years of coeliac disease diagnosis or in undiagnosed coeliacs.<ref name="pmid16979946">Al-Toma A, Goerres MS, Meijer JW, et al. (2006). [Expression error: Missing operand for > "Cladribine therapy in refractory celiac disease with aberrant T cells"]. Clin. Gastroenterol. Hepatol. 4 (11): 1322–7; quiz 1300. doi:10.1016/j.cgh.2006.07.007. PMID 16979946. </ref><ref name="pmid16527694">Al-Toma A, Goerres MS, Meijer JW, Peña AS, Crusius JB, Mulder CJ (2006). [Expression error: Missing operand for > "Human leukocyte antigen-DQ2 homozygosity and the development of refractory celiac disease and enteropathy-associated T-cell lymphoma"]. Clin. Gastroenterol. Hepatol. 4 (3): 315–9. doi:10.1016/j.cgh.2005.12.011. PMID 16527694. </ref> 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<ref name="pmid17410290">Al-Toma A, Verbeek WH, Mulder CJ (2007). [Expression error: Missing operand for > "Update on the management of refractory coeliac disease"]. Journal of gastrointestinal and liver diseases : JGLD 16 (1): 57–63. PMID 17410290. </ref> 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.<ref name="pmid15696854">Meijer JW, Mulder CJ, Goerres MG, Boot H, Schweizer JJ (2004). [Expression error: Missing operand for > "Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment"]. Scand. J. Gastroenterol. Suppl. 39 (241): 78–84. doi:10.1080/00855920410014605. PMID 15696854. </ref>

See also

References

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