Lee Tae Yeong, Han Sang Young, Moon Sung Hun, Jang Chae Ryung, Jang Jin Seok, Park Mi Kyoung, Lee Jong Hun, Roh Myung Hwan, Shin Woo Won, Choi Seok Ryeol
Department of Internal Medicine, Dong-A University College of Medicine, Busan, Korea.
Korean J Gastroenterol. 2004 Feb;43(2):133-6.
A 68-year-old woman with known severe aortic stenosis was admitted to the hospital because of hematochezia and dizziness. She had received several blood transfusions over the preceding 3 years and undergone right hemicolectomy 2 years ago for severe lower gastrointestinal bleeding. Postoperative histology revealed angiodysplasia involving the ascending colon. After the hemicolectomy, she continued to have hematochezia and anemia and required additional blood transfusions for anemia. During this admission, platelet count, activated partial-thromboplastin time, von Willebrand factor antigen, and von Willebrand factor ristocetin cofactor were normal. She had a severe deficiency of high-molecular-weight multimers of von Willebrand factor. Colonoscopy showed angiodysplasia in the transverse colon at this time. Successful coagulation of the bleeding angiodysplasia was achieved by argon plasma coagulator. No additional bleeding was observed thereafter. We report a case of Heyde's syndrome with abnormal von Willebrand factor in a patient who presented with intestinal angiodysplasia and aortic stenosis.
一名68岁患有严重主动脉瓣狭窄的女性因便血和头晕入院。在过去3年中,她接受了多次输血,并于2年前因严重下消化道出血接受了右半结肠切除术。术后组织学检查显示升结肠存在血管发育异常。半结肠切除术后,她仍有便血和贫血,因贫血需要额外输血。此次入院期间,血小板计数、活化部分凝血活酶时间、血管性血友病因子抗原和血管性血友病因子瑞斯托霉素辅因子均正常。她存在严重的血管性血友病因子高分子量多聚体缺乏。此时结肠镜检查显示横结肠存在血管发育异常。通过氩等离子体凝固器成功凝固了出血的血管发育异常部位。此后未观察到再次出血。我们报告了一例患有肠道血管发育异常和主动脉瓣狭窄的患者出现血管性血友病因子异常的海德综合征病例。