This entity occurs most often in the background of coeliac disease usually at an adult age. The diagnosis is determined not uncommonly by explorative laparotomy based in subileus or intestinal perforation. Lesions start within the epithelium.
The prognosis is poorer than for other T-cell lymphomas.
The lesions are often multifocal in the jejunum and/or ileum. Surgery is therefore insufficient as the only treatment form. Perforation of the small intestine is a danger at the start of chemotherapy.