Horai Yoshiro, Miyamura Tomoya, Hirata Akie, Nakamura Masataka, Takahama Soichiro, Ando Hitoshi, Minami Rumi, Yamamoto Masahiro, Suematsu Eiichi
Department of Internal Medicine and Rheumatology, Clinical Research Center, National Hospital Organization Kyushu Medical Center, Fukuoka.
Intern Med. 2010;49(18):2013-6. doi: 10.2169/internalmedicine.49.3834. Epub 2010 Sep 15.
We report a 29-year-old woman with a 2.5 year history of mixed connective tissue disease (MCTD) who developed idiopathic portal hypertension (IPH) and thrombocytopenia as a result of hypersplenism. She had recurrent esophagogastric variceal rupture. Hematological examination also revealed low levels of protein C activity. The liver biopsy specimen showed non-specific mild inflammation and no thrombi. However, portal vein thrombosis developed after splenectomy. This was a rare case of severe complications of IPH accompanying MCTD and protein C deficiency.
我们报告一名29岁女性，患有混合性结缔组织病（MCTD）2.5年，因脾功能亢进发展为特发性门静脉高压（IPH）和血小板减少症。她反复出现食管胃静脉曲张破裂。血液学检查还显示蛋白C活性水平较低。肝脏活检标本显示非特异性轻度炎症，无血栓形成。然而，脾切除术后发生了门静脉血栓形成。这是一例罕见的IPH严重并发症伴MCTD和蛋白C缺乏症的病例。
