发病机制
降纤酶
医学
疾病
免疫学
细胞因子
干细胞
移植
肝静脉闭塞性疾病
生物信息学
内科学
病理
造血干细胞移植
生物
遗传学
作者
Jason Coppell,Simon Brown,David J. Perry
出处
期刊:Blood Reviews
[Elsevier BV]
日期:2003-04-04
卷期号:17 (2): 63-70
被引量:98
标识
DOI:10.1016/s0268-960x(03)00002-x
摘要
Hepatic veno-occlusive disease (VOD) is a major cause of morbidity and mortality following high dose cytotoxic therapy for stem cell transplantation (SCT). Pre-existing liver damage, SCT-related therapy, and genetic polymorphisms all appear to increase the risk of developing VOD. Studies of biological markers during SCT suggest that cytokines, haemostasis, and hepatic drug metabolism via the glutathione pathway are all involved in the pathogenesis of VOD. Until recently, treatment options were limited and experimental therapies directed at the pathogenesis of the disease were mostly unsuccessful. However, Defibrotide, a relatively new agent that has modulatory effects on vascular endothelium, cytokine release, and haemostasis, has been used with some success in the management and prophylaxis of VOD. In the future, a better understanding of genetic polymorphisms and biological markers which may be important in the pathogenesis of VOD, may enable us to predict which patients are most likely to be affected.
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