Enteropathy-associated T-cell lymphoma: from indolent to aggressive.

Abstract

Enteropathy-associated T-cell lymphoma (EATL) is a rare neoplasm that accounts for <5% of peripheral T-cell lymphomas. EATL is derived from malignant transformation of intestinal intraepithelial lymphocytes and is the most common neoplastic complication of celiac disease. It is rare in patients diagnosed at a young age, although in long-standing coeliac disease it may be the debut of the disease. Its diagnosis involves imaging techniques, biopsies and immunohistochemistry, the main tool needed being a high diagnostic suspicion. The prognosis is poor and there is currently no consensus on the best therapeutic approach.

Keywords: T-lymphoma, celiac disease, indolent.