Enteropathy associated T-cell lymphoma: a review of diagnostic findings and therapeutic strategies

Enteropathy associated T-cell lymphoma (EATL) is an uncommon type of non-Hodgkin T-cell lymphoma of the intestinal intraepithelial T lymphocytes. Frequently presenting as intestinal perforation, ulceration, or a mass in the jejunum and proximal ileum in the gastrointestinal tract, EATL carries a poor prognosis with absence of well-defined therapeutic protocols for management. The WHO recognizes two distinct variants of EATL, the type I/classical variant and type II/monomorphic variant, based on different clinical features and histopathologic findings. EATL type I has a higher incidence in geographic regions with a high prevalence of celiac disease such as Northern Europe and is rare in the Asian population, in whom celiac disease is also rare. EATL type II can occur sporadically, has a higher incidence in Asian populations, with no definite association with celiac disease. This review aims to summarize the clinicopathologic findings of EATL and current approaches to management.