Enteropathy-associated T-cell lymphoma
| Enteropathy-associated T-cell lymphoma | |
|---|---|
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| Micrograph of enteropathy-associated T cell lymphoma (upper right of image). H&E stain. | |
| Classification and external resources | |
| Specialty | oncology |
| ICD-10 | C86.2 |
| ICD-O | M9717/3 |
Enteropathy-associated T-cell lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell lymphoma that affects 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). These cancerous T-cells are a possible consequence of refractory cases of coeliac disease or in chronic, untreated cases in genetically susceptible individuals.
EATL is most frequent in Europe, where it represents 9.4% of all peripheral T cell lymphomas. Association with celiac disease is consistently demonstrated in only 30% of patients. The global incidence of this lymphoma is rare, being about 0.5 to 1 per million.
EATL can be classified as an extranodal peripheral T cell lymphoma, a category it shares with hepatosplenic T cell lymphoma and panniculitic T cell lymphoma. It can be further classified in type I and II EATL.
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. (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-gliadin to T-cells, constant over-stimulation of T-cell eventually results in neoplastic growth. EATL typically appears after the 4th decade of life, within 3 years of coeliac disease diagnosis or in undiagnosed coeliacs. 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. 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.
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