Primary upper small-intestinal lymphoma

Primary upper small-intestinal lymphoma (PUSIL) has now been recognized as a distinct clinical entity with a distinct geographic distribution. Herewith are presented 40 cases of PUSIL seen at Pahlavi University Hospitals in Southern Iran. The investigation reveals the lymphoma to be predominantly a disease of those under 30 and males; the major complaints and physical findings point to an intraabdominal disease. An exception has been clubbing and osteoarthropathy. Special features of PUSIL include: (1) protein loss into the gastro-intestinal tract leading to hypoalbuminemia and edema; (2) an antibiotic-responsive diarrhea and steatorrhea; and (3) an associated abnormal heavy-chain protein. The study further stresses the importance of peroral small-intestinal biopsy and the pathologic features of this condition.