Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome (SOS) is a rare complication characterized by hepatomegaly, right-upper quadrant pain, jaundice, and ascites, occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) and, less commonly, other conditions. We review pathogenesis, clinical appearance and diagnostic criteria, risk factors, prophylaxis, and treatment of the VOD occurring post-HSCT. The injury of the sinusoidal endothelial cells with loss of wall integrity and sinusoidal obstruction is the basis of development of postsinusoidal portal hypertension responsible for clinical syndrome. Risk factors associated with the onset of VOD and diagnostic tools have been recently updated both in the pediatric and adult settings and here are reported. Treatment includes supportive care, intensive management, and specific drug therapy with defibrotide. Because of its severity, particularly in VOD with associated multiorgan disease, prophylaxis approaches are under investigation. During the last years, decreased mortality associated to VOD/SOS has been reported being it attributable to a better intensive and multidisciplinary approach.

中文翻译：

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同种异体造血干细胞移植后VOD / SOS的诊断和治疗。

肝静脉闭塞性疾病（VOD）或正弦窦阻塞综合征（SOS）是一种罕见的并发症，其特征是大剂量化疗，造血干细胞移植（HSCT）后发生肝肿大，右上腹痛，黄疸和腹水。不太常见的是其他条件。我们回顾了HSCT后发生的VOD的发病机理，临床表现和诊断标准，危险因素，预防和治疗。窦壁内皮细胞的损害以及壁完整性的丧失和窦壁梗阻是引起临床综合征的窦后门静脉高压症发展的基础。与VOD发作和诊断工具有关的危险因素最近在儿科和成人环境中均已更新，并在此进行了报道。治疗包括支持性护理，强化管理，以及使用去纤蛋白特异的药物治疗。由于其严重性，特别是在伴有多器官疾病的VOD中，正在研究预防方法。在过去的几年中，已经报道了与VOD / SOS相关的死亡率降低，这归因于更好的集约化和多学科方法。