Stoneham Sara, Lennard Lynne, Coen Pietro, Lilleyman John, Saha Vaskar
Cancer Research UK, Children's Cancer Group, Bart's and The London, Queen Mary School of Medicine and Dentistry, London, UK.
Br J Haematol. 2003 Oct;123(1):100-2. doi: 10.1046/j.1365-2141.2003.04578.x.
The case records of 99 consecutive children with acute lymphoblastic leukaemia who received either 6-thioguanine (6-TG) or 6-mercaptopurine (6-MP) as maintenance therapy for at least 1 year were reviewed for hepatic veno-occlusive disease (VOD). Overall, 12% of those on 6-TG developed VOD (all boys). Isolated persistent thrombocytopenia appeared to be the earliest indicator of incipient VOD. Multivariate analysis identified male sex and 6-TG as risk factors. In all cases, VOD was mild and reversible on withdrawing 6-TG or replacing it with 6-MP. The data implicate a sex-linked polymorphic variation in xenobiotic pathways of thiopurine metabolism in the pathogenesis of VOD.
对99例接受6-硫鸟嘌呤（6-TG）或6-巯基嘌呤（6-MP）维持治疗至少1年的急性淋巴细胞白血病患儿的病例记录进行了回顾，以研究肝静脉闭塞病（VOD）。总体而言，接受6-TG治疗的患儿中有12%发生了VOD（均为男孩）。孤立性持续性血小板减少似乎是早期VOD的最早指标。多因素分析确定男性性别和6-TG为危险因素。在所有病例中，VOD均较轻，停用6-TG或用6-MP替代后可逆转。数据表明，硫嘌呤代谢的外源性途径中与性别相关的多态性变异在VOD的发病机制中起作用。
