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Monomorphic epitheliotropic intestinal T-cell lymphoma

ORPHA:652658· ICD-10 C86.2

Definition

A rare T-cell non-Hodgkin lymphoma characterized by monomorphic cytomorphology and epitheliotropism. It is mostly detected in the small intestine but can also be present in the colon, duodenum orand stomach. It is an aggressive tumor that can disseminate to mesenteric lymph nodes, lung, liver, brain and skin. Major clinical features include abdominal pain, gastrointestinal bleeding, obstruction or perforation, diarrhoea, and weight loss. It is not associated to coeliac disease.