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血液系统恶性肿瘤患儿异基因骨髓移植后肝静脉闭塞病:发病率、发病时间及危险因素

Veno-occlusive disease of the liver after allogeneic bone marrow transplantation in children with hematologic malignancies: incidence, onset time and risk factors.

作者信息

Hasegawa S, Horibe K, Kawabe T, Kato K, Kojima S, Matsuyama T, Hirabayashi N

机构信息

Department of Pediatrics, Nagoya University School of Medicine, Japan.

出版信息

Bone Marrow Transplant. 1998 Dec;22(12):1191-7. doi: 10.1038/sj.bmt.1701506.

DOI:10.1038/sj.bmt.1701506
PMID:9894723
Abstract

One hundred and forty children with hematologic malignancies undergoing allogeneic BMT were reviewed in order to clarify the incidence, onset time, and risk factors for veno-occlusive disease (VOD) of the liver. Thirty-eight patients (27.1%) developed VOD diagnosed according to the Seattle clinical criteria. Seventeen patients developed VOD within 20 days of transplantation (early-onset) and in 21 patients developed after day 20 (late-onset) including eight patients with histological confirmation. Late-onset VOD occurred from day 21 to day 508 (median day 39). Moderate or severe VOD developed in 11 early-onset and 13 late-onset patients. Death occurred in eight early-onset and 10 late-onset patients. Serum albumin and cholinesterase levels prior to the start of pretransplant conditioning were significantly lower in early-onset VOD than in late-onset VOD. Multivariate analysis showed that low serum albumin levels (< or =3.7 g/dl) prior to the start of pretransplant conditioning was most strongly associated with the development of VOD. Donor mismatch (other than HLA-matched relatives), use of minocycline, and a long interval (> or =13 months) between diagnosis and BMT were also significantly associated with the development of VOD. In contrast, use of fosfomycin was associated with a decreased risk. Our data suggest that hepatic function reserve is important in the development and onset time of VOD. Veno-occlusive disease of the liver is a complication which may occur a long time after transplantation.

摘要

为明确肝脏静脉闭塞性疾病(VOD)的发病率、发病时间及危险因素,对140例接受异基因骨髓移植(BMT)的血液系统恶性肿瘤患儿进行了回顾性研究。38例患者(27.1%)发生了符合西雅图临床标准诊断的VOD。17例患者在移植后20天内发生VOD(早发型),21例患者在移植后20天之后发生(晚发型),其中8例经组织学证实。晚发型VOD发生于移植后第21天至第508天(中位时间为第39天)。11例早发型和13例晚发型患者发生了中度或重度VOD。8例早发型和10例晚发型患者死亡。移植前预处理开始前,早发型VOD患者的血清白蛋白和胆碱酯酶水平显著低于晚发型VOD患者。多因素分析显示,移植前预处理开始前低血清白蛋白水平(≤3.7 g/dl)与VOD的发生最密切相关。供体不匹配(非HLA匹配亲属)、使用米诺环素以及诊断与BMT之间间隔时间长(≥13个月)也与VOD的发生显著相关。相比之下,使用磷霉素与风险降低相关。我们的数据表明,肝功能储备在VOD的发生及发病时间方面很重要。肝脏静脉闭塞性疾病是一种可能在移植后很长时间发生的并发症。

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