Komatsu Yoshimichi, Koizumi Tomonobu, Yasuo Masanori, Urushihata Kazuhisa, Yamamoto Hiroshi, Hanaoka Masayuki, Kubo Keishi, Kawakami Satoshi, Honda Takayuki, Fujimoto Keisaku, Hachiya Tsutomu
The First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Intern Med. 2010;49(12):1149-53. doi: 10.2169/internalmedicine.49.3148. Epub 2010 Jun 15.
A 46-year-old woman was admitted to our hospital because of pain in the right upper quadrant and dyspnea. Abdominal and chest computed tomography (CT) scans revealed areas of low attenuation in both hepatic lobes, left pleural effusion, and multiple nodules in both lungs. Laboratory data indicated disseminated intravascular coagulation. She developed rapidly progressive respiratory and hepatic failure despite intensive treatment including mechanical ventilation and died of respiratory failure 3 weeks after admission. Immunohistochemical analysis of liver necropsy and cytology of the left plural effusion stained positive for factor VIII-related antigen and CD31. Based on these observations, a diagnosis of hemangioendothelioma (EHE), a rare vascular tumor, was made. A rapid clinical course and fatal outcome, as in the present case, are rare clinical manifestations in EHE.
一名46岁女性因右上腹疼痛和呼吸困难入住我院。腹部和胸部计算机断层扫描（CT）显示肝两叶有低密度区、左侧胸腔积液以及双肺多发结节。实验室数据提示弥散性血管内凝血。尽管接受了包括机械通气在内的强化治疗，她仍迅速出现进行性呼吸和肝功能衰竭，并在入院3周后死于呼吸衰竭。肝尸检的免疫组织化学分析和左侧胸腔积液的细胞学检查显示，VIII因子相关抗原和CD31呈阳性。基于这些观察结果，诊断为血管内皮瘤（EHE），一种罕见的血管肿瘤。如本例所示，快速的临床病程和致命结局是EHE罕见的临床表现。
