Akutagawa Takashi, Shindo Takero, Yamanouchi Kohei, Hayakawa Masaki, Ureshino Hiroshi, Tsuruoka Nanae, Sakata Yasuhisa, Shimoda Ryo, Noguchi Ryo, Furukawa Kojiro, Morita Shigeki, Iwakiri Ryuichi, Kimura Shinya, Matsumoto Masanori, Fujimoto Kazuma
Department of Internal Medicine and Gastrointestinal Endoscopy, Saga University School of Medicine, Japan.
Department of Hematology, Respiratory Medicine and Oncology, Saga University School of Medicine, Japan.
Intern Med. 2017 Sep 15;56(18):2431-2433. doi: 10.2169/internalmedicine.8603-16. Epub 2017 Aug 21.
A 67-year-old woman with recurrent gastrointestinal bleeding and aortic valve stenosis presented with spurting bleeding from angiodysplasia of the upper jejunum. As electrophoresis revealed decreased levels of high-molecular-weight (HMW) von Willebrand factor (VWF) multimers, she was diagnosed with Heyde's syndrome. After aortic valve replacement, her HMW VWF levels quickly recovered to normal, and the gastrointestinal bleeding ceased. However, capsule endoscopy still revealed gastrointestinal angiodysplasia six months later. This case shows that minute analyses of VWF multimers enable the diagnosis and confirmation of the resolution of Heyde's syndrome, and implies that gastrointestinal angiodysplasia can be attributed to unknown factors other than decreased VWF multimers.
一名67岁患有复发性胃肠道出血和主动脉瓣狭窄的女性，出现了来自空肠上段血管发育异常的喷射性出血。由于电泳显示高分子量（HMW）血管性血友病因子（VWF）多聚体水平降低，她被诊断为海德综合征。主动脉瓣置换术后，她的HMW VWF水平迅速恢复正常，胃肠道出血停止。然而，六个月后胶囊内镜仍显示有胃肠道血管发育异常。该病例表明，对VWF多聚体的细致分析有助于海德综合征的诊断和解决情况的确认，并暗示胃肠道血管发育异常可能归因于VWF多聚体减少以外的未知因素。
