Treatment of Enteropathy-Associated and Monomorphic Epitheliotropic Intestinal T-Cell Lymphomas

Enteropathy-associated T-cell lymphoma (EATL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) are rare subsets of peripheral T-cell lymphoma (PTCL) which together account for less than 5% of PTCL. EATL (sometimes referred to as type 1 EATL) is a complication of celiac disease and is more common in Europe. MEITL (sometimes referred to as type 2 EATL) is not associated with celiac disease and is more common in Asia. SETD2 pathogenic variants and, to a lesser extent, STAT5B pathogenic variants are seen in most cases of EATL and MEITL.[1,2] Because a gluten-free diet prevents the development of lymphoma in patients with celiac disease, patients diagnosed in childhood rarely develop EATL. The diagnosis of celiac disease is usually made by finding villous atrophy in the resected intestine. Surgery is often required for diagnosis and to avoid perforation during therapy.[3]