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 pat hogenesis of this association remains to be clarified in most cases. S even unusual cases of angiocentric T-cell lymphoma of the intestine pr esenting with bowel ischemia or perforation are reported. Their clinic opathologic features were analyzed. Methods: Clinical records and hist opathology were reviewed. Immunophenotypic studies and EBER1 in situ h ybridization for Epstein-Barr virus were performed. Results: All patie nts (3 men and 4 women; mean age, 42 years) presented with acute abdom inal pain, bloody stool, or bowel perforation. The intestinal lesions were ulcerated and transmurally necrotic or gangrenous. The angiodestr uction of the tumor cells appeared to be responsible for the presentin g 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. Conclus ions: Intestinal angiocentric T-cell lymphoma represents a distinct et iology of bowel ischemia. The condition should be taken into considera tion, particularly in young adults with ischemic bowel disease.