异基因造血干细胞移植后肝静脉闭塞病的防治效果分析

背景与目的:肝静脉闭塞病(veno-occlusive disease of the liver,VOD)是造血干细胞移植常见的并发症之一,重型VOD的病死率几乎达100%,目前VOD的预防和治疗仍较困难。本研究总结具有发生VOD高危因素的造血干细胞移植患者的VOD预防和治疗方案,探讨更好的防治方法。方法:1999～2005年在中山大学附属第三医院用马利兰和环磷酰胺预处理的18例异基因造血干细胞移植患者,在移植前给予前列腺素E1、还原型谷胱甘肽、门冬氨酸鸟氨酸预防VOD,移植后发生VOD的患者予前列腺素E1、低分子肝素、组织型纤溶酶原激活物(tissue plasminogen activator,tPA)治疗。结果:18例患者中有11例移植后出现谷丙转氨酶水平升高,经治疗后降至正常;仅有1例发生VOD,为重型,经前列腺素E1和低分子肝素治疗效果不佳,予tPA治疗后完全缓解,副作用为皮肤粘膜出血。结论:针对VOD发病机制的多个方面,联合应用前列腺素E1、还原型谷胱甘肽、门冬氨酸鸟氨酸预防VOD,可能起到较好的预防作用,使用安全方便,值得进一步研究推广;在目前条件下对重型VOD,在严密观察下使用tPA可作为一个治疗选择。

Efficiency of prevention and treatment of veno-occlusive disease of the liver after allogeneic stem cell transplantation

BACKGROUND & OBJECTIVE: Veno-occlusive disease of the liver (VOD) is one of the most common complications in patients after hematopoietic stem cell transplantation (HSCT). So far, the mortality of severe VOD is almost 100%. How to deal with the disease remains to be an unresolved problem. This study was to explore more efficient methods of prevention and treatment of VOD. METHODS: Clinical data of 18 patients undergoing allogeneic HSCT were analyzed. All the patients were pretreated with busulfan and cyclophosphamide, and treated with prostaglandin E1, glutathione and ornithine aspartate before HSCT to prevent VOD. Prostagland E1, low molecular weight heparin and tissue plasminogen activator (tPA) were used to treat the patient with VOD. RESULTS: The serum level of glutamate-pyruvate transaminase was elevated in 11 patients after HSCT, and all the patients were cured. Only 1 patient suffered VOD which was severe. Prostaglandin E1 and low molecular weight heparin were not effective in this case, while tPA was. The side effect of tPA was bleeding of the skin and mucosa. CONCLUSIONS: The combination of prostaglandin E1, glutathione and ornithine aspartate is probably effective and safe in preventing VOD for it aims at many aspects of pathogenesis of VOD. tPA can be used carefully as a choice of treating severe VOD.