Heikinheimo M, Halila R, Fasth A
Children's Hospital, University of Helsinki, Finland.
Blood. 1994 May 15;83(10):3036-40.
Veno-occlusive disease of the liver (VOD) is a life-threatening complication occurring in patients undergoing bone marrow transplantation (BMT). Although clinical signs and laboratory parameters such as elevation of serum bilirubin often suggest this condition, it would be useful to identify early biochemical markers for VOD. Fibrous alterations in the hepatic venules and small lobular veins occur during development of VOD; these changes are accompanied by the deposition of types I and III collagen in the liver tissue. Since the N-terminal propeptide of type III procollagen (PIIINP) is a sensitive marker of liver and lung fibrosis, we undertook a study to evaluate the usefulness of measurements of serum PIIINP in children with VOD. Seven of the 28 children who underwent BMT, both allogenic and autologous, developed VOD. All seven had an increase of more than 100 ng/mL in the serum PIIINP level, whereas only one of the remaining 21 children not affected by VOD had an increment of PIIINP more than 100 ng/mL (P = .0001). The levels of serum PIIINP were higher in the VOD group during the follow-up period of up to 91 days after BMT. The elevation of PIIINP also occurred at a stage of the disease usually preceding any other laboratory or clinical signs of VOD. Serum concentration of PIIINP thus seems to be of value as an early marker for VOD in children undergoing BMT.
肝静脉闭塞病（VOD）是接受骨髓移植（BMT）患者中出现的一种危及生命的并发症。尽管临床体征和实验室参数如血清胆红素升高常常提示这种情况，但确定VOD的早期生化标志物将很有帮助。在VOD发生过程中，肝小静脉和小叶内小静脉会出现纤维性改变；这些变化伴随着I型和III型胶原在肝组织中的沉积。由于III型前胶原N端前肽（PIIINP）是肝和肺纤维化的敏感标志物，我们开展了一项研究来评估检测血清PIIINP对VOD患儿的作用。接受同种异体和自体BMT的28名儿童中，有7名发生了VOD。所有7名患儿血清PIIINP水平升高超过100 ng/mL，而其余21名未受VOD影响的儿童中只有1名PIIINP升高超过100 ng/mL（P = .0001）。在BMT后长达91天的随访期内，VOD组的血清PIIINP水平更高。PIIINP升高也发生在疾病的一个阶段，通常先于VOD的任何其他实验室或临床体征出现。因此，血清PIIINP浓度似乎可作为接受BMT儿童VOD的早期标志物。
