Inoue Ai, Michitaka Kojiro, Shigematsu Shuichiro, Konishi Ichiro, Hirooka Masashi, Hiasa Yoichi, Matsui Hidetaka, Matsuura Bunzo, Horiike Norio, Hato Takaaki, Miyaoka Hiroaki, Onji Morikazu
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city.
Intern Med. 2007;46(14):1095-100. doi: 10.2169/internalmedicine.46.6438. Epub 2007 Jul 17.
A 27-year-old man was admitted due to abdominal fullness. He had ascites and subcutaneous nodules on his head, with liver dysfunction and eosinophilia. Abdominal imaging revealed obstruction of the hepatic veins and stenosis of the inferior vena cava. Histological diagnosis of a subcutaneous nodule revealed obstructive thrombophlebitis with eosinophils. Tyrosine kinase created by fusion of the FIP1L1 and PDGFRA genes, which is characteristic of hypereosinophilic syndrome (HES), was detected. He was diagnosed with Budd-Chiari syndrome associated with HES. Liver function tests improved after interventional therapy followed by steroid therapy. It is important to diagnose the cause of Budd-Chiari syndrome.
一名27岁男性因腹部胀满入院。他有腹水，头部有皮下结节，伴有肝功能障碍和嗜酸性粒细胞增多。腹部影像学检查显示肝静脉阻塞和下腔静脉狭窄。皮下结节的组织学诊断为伴有嗜酸性粒细胞的阻塞性血栓性静脉炎。检测到由FIP1L1和PDGFRA基因融合产生的酪氨酸激酶，这是高嗜酸性粒细胞综合征（HES）的特征。他被诊断为与HES相关的布加综合征。介入治疗后再进行类固醇治疗，肝功能检查结果有所改善。诊断布加综合征的病因很重要。
