Nasr K, Haghighi P, Bakhshandeh K, Abadi P, Lahimgarzadeh A
Am J Dig Dis. 1976 Apr;21(4):313-23. doi: 10.1007/BF01071844.
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 gastrointestinal 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.
原发性上消化道小肠淋巴瘤(PUSIL)现已被确认为一种具有独特地理分布的独特临床实体。本文介绍了在伊朗南部巴列维大学医院所见的40例PUSIL病例。调查显示,该淋巴瘤主要是30岁以下人群及男性的疾病;主要症状和体格检查结果表明是一种腹腔内疾病。杵状指和骨关节炎是个例外。PUSIL的特殊特征包括:(1)蛋白质丢失到胃肠道导致低白蛋白血症和水肿;(2)对抗生素有反应的腹泻和脂肪泻;(3)相关的异常重链蛋白。该研究进一步强调了经口小肠活检的重要性以及这种疾病的病理特征。