A case report of NK/T cell lymphoma arising primarily in small intestine in a young patient

Abstract

A 28-year-old male was referred to our hospital for fever of unknown origin (FUO). He had histories of FUOs at the age of 23 and 25. After the admission he experienced tarry stools, then endoscopic examination was performed and found multiple ulcerations in the small intestine. Because anemia was refractory, partial resection of intestine was performed. Histological examination of the resected specimen showed angiocentric proliferation of atypical cells with irregular nuclear contour. They were CD3+, CD56-, TIA1+, TCRβ+, TCRγ- by immunohistochemistry and EBER-ISH+. There was no other lesion except duodenum, jejunum, and regional mesenteric lymph node and then the diagnosis of primary intestinal NK/T cell lymphoma of T-cell lineage was rendered. Five courses of cyclophosphamide, hydroxydaunorubicin, vincris-tine, and prednisolone were performed. Although the lesions became scar endoscopically, scattered CD3+, EBER+ cells persisted to be detected microscopically. Additional cord blood transplantation was performed twice, but the patient died because of pneumonitis and encephalitis 17 months after the initial diagnosis. Primary intestinal NK/T cell lymphoma is a rare and highly aggressive disease in spite of intensive chemotherapy. We report this case with a review of the literature.