Clinique des Maladies de l'Appareil Digestif et de la Nutrition, Hôpital Claude Huriez, Rue Michel Polonovski, 59037 Lille Cedex, France.
Nat Rev Gastroenterol Hepatol. 2010 Jul;7(7):410-4. doi: 10.1038/nrgastro.2010.59. Epub 2010 May 4.
A 33-year-old female presented with a history of recurrent epigastric pain and vomiting of bile, accompanied by duodenal wall thickening seen on an abdominal CT scan and a congestive, bluish and ischemic-looking duodenal mucosa as visualized by endoscopy. The patient's medical history was notable for paroxysmal nocturnal hemoglobinuria diagnosed 15 years before. The patient was usually treated with danazol and warfarin and she received regular blood transfusions for hemolytic anemia.
Medical history and physical examination. Blood tests, coagulation study, search for thrombophilic disorders and small vessel vasculitis. Stool culture and virology. Test for autoantibodies. Abdominal CT scan. Upper gastrointestinal endoscopy with duodenal biopsies.
Small bowel ischemia complicating paroxysmal nocturnal hemoglobinuria.
Treatment with warfarin was started but ischemic episodes recurred despite appropriate anticoagulation. Treatment with the complement inhibitor eculizumab was then proposed.
一名 33 岁女性因反复发作性上腹痛和胆汁呕吐就诊，腹部 CT 扫描显示十二指肠壁增厚，内镜下可见充血、蓝紫色和缺血样十二指肠黏膜。患者的病史有 15 年前诊断为阵发性睡眠性血红蛋白尿症。患者通常接受达那唑和华法林治疗，因溶血性贫血接受定期输血。
详细病史和体格检查。血液检查、凝血研究、血栓形成倾向和小血管血管炎的检查。粪便培养和病毒学检查。自身抗体检测。腹部 CT 扫描。上消化道内镜检查和十二指肠活检。
阵发性睡眠性血红蛋白尿症并发小肠缺血。
开始使用华法林治疗，但尽管抗凝治疗恰当，缺血发作仍反复发生。随后提出使用补体抑制剂依库珠单抗治疗。
