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WHO Classification of Tumours
Enteropathy-associated T-cell lymphoma
Small intestine


Definition

Enteropathy-associated intestinal T-cell lymphoma (EATL) is an intestinal tumour of intraepithelial T-lymphocytes composed of large, often polymorphic lymphoid cells, which are negative for CD56. It is also referred to as Type I EATL. The disease is uncommon in most parts of the world, but is seen with increasing frequency in areas with a high prevalence of coeliac disease, e.g. Northern Europe. Most patients with EATL show adult-onset disease or are diagnosed as having coeliac disease in the same clinical episode in which the lymphoma is diagnosed. Patients present with abdominal pain, most often as emergencies associated with intestinal perforation and/or obstruction. Histologically, the degree of infiltration is highly variable. Prognosis is usually poor, with death frequently resulting from abdominal complications in patients already weakened by uncontrolled malabsorption, but long-term survivals are recorded
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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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