Enteropathy-Associated T-Cell Lymphoma: Epidemiology, Natural History, and Management in the Current Era

Celiac disease (CeD) is a chronic autoimmune enteropathy which is seen in genetically predisposed individuals (HLA DQ2 and HLA DQ8 positive) as a result of intolerance to dietary gluten. The mortality in CeD is due to refractory celiac disease (RCD) and enteropathy-associated T-cell lymphoma (EATL). EATL is a rare entity accounting only up to less than 5% of the primary gastrointestinal lymphomas and is found in the older age group (60–70 years) with most of the cases reported from Europe. A strong association has been found between CeD and EATL, in particular RCD2. Diagnosis of EATL is confirmed by histopathology and immunophenotyping of representative biopsies from intestine. Staging is done with computed tomography (CT) or FDG-PET/CT imaging. Most of patients are diagnosed in advanced stage and not suitable for chemotherapy at time of diagnosis. Treatment is surgery and chemotherapy followed by autologous stem cell therapy. Anthracycline-based CHOP regimen is most commonly used, and brentuximab vedotin can be added in CD30 positive patients. It has aggressive course and very poor outcome with all the currently available therapies for peripheral T-cell Lymphoma. Target therapies or recruiting more patients in clinical trials may give some better outcome in future. Early detection of celiac disease by implementation of common surveillance may improve outcome.