Enteropathy-associated T-cell lymphoma epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Enteropathy-associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell non-hodgkin lymphoma that affects the small intestine, it is composed of large lymphoid cells. Enteropathy-associated T-cell lymphoma has two subtypes, type I enteropathy-associated T-cell lymphoma which has a strong association with celiac disease and it is more common in western countries and type II enteropathy-associated T-cell lymphoma which is mostly found among the Asian population. Genes involved in the pathogenesis of this disease include 8q24, T-cell receptor (TCR) beta and gamma, and 16q genes. On gross pathology, multiple intestinal ulcers are characteristic findings of EATL. On microscopic histopathological analysis, monotonous cells, round or angulated vesicular nuclei, and prominent nucleoli are characteristic findings of enteropathy-associated T-cell lymphoma. There are no established causes for enteropathy-associated T-cell lymphoma. EATL must be differentiated from other diseases such as peptic ulcer, poorly-differentiated adenocarcinoma, MALT lymphoma, diffuse large B cell lymphoma, and mantle cell lymphoma.

Epidemiology and Demographics

• Enteropathy-associated T cell lymphoma is a very rare form of extranodal non-Hodgkin lymphoma, with an average incidence of 0.10 to 1.5 per 100,000 inhabitants per year, mostly occurs in the older adult, peak incidence average of 59 years old and involves proximal small intestine (duodenum and jejunum).
• Type II EATL is more common in the Asian population and not related to celiac disease, whereas type I EATL is more frequently happens in western Europe.