Angiocentric T-cell lymphoma of the intestine: A distinct etiology of ischemic bowel disease

Background & Aims: Bowel ischemia and perforation is an infrequent but ominous event in patients with malignant lymphoma. The underlying pathogenesis of this association remains to be clarified in most cases. Seven unusual cases of angiocentric T-cell lymphoma of the intestine presenting with bowel ischemia or perforation are reported. Their clinicopathologic features were analyzed. Methods: Clinical records and histopathology were reviewed. Immunophenotypic studies and EBER1 in situ hybridization for Epstein-Barr virus were performed. Results: All patients (3 men and 4 women; mean age, 42 years) presented with acute abdominal pain, bloody stool, or bowel perforation. The intestinal lesions were ulcerated and transmurally necrotic or gangrenous. The angiodestruction of the tumor cells appeared to be responsible for the presenting bowel ischemia. The neoplastic cells expressed a pan-T-cell antigen CD45RO; 4 of them contained Epstein-Barr virus transcripts EBER1. The prognosis was grave; 6 patients died within 3 months of onset. Conclusions: Intestinal angiocentric T-cell lymphoma represents a distinct etiology of bowel ischemia. The condition should be taken into consideration, particularly in young adults with ischemic bowel disease.