Paediatric Clinic, National University Hospital Rigshospitalet, Blegdamsvej 9, Copenhagen, Denmark.
Bone Marrow Transplant. 2012 Aug;47(8):1020-9. doi: 10.1038/bmt.2011.161. Epub 2011 Aug 29.
Bronchiolitis obliterans (BO) following allogeneic haematopoietic SCT (HSCT) is a serious complication affecting 1.7-26% of the patients, with a reported mortality rate of 21-100%. It is considered a manifestation of chronic graft-versus-host disease, but our knowledge of aetiology and pathogenesis is still limited. Diagnostic criteria are being developed, and will allow more uniform and comparable research activities between centres. At present, no randomised controlled trials have been completed that could demonstrate an effective treatment. Steroids in combination with other immunosuppressive drugs still constitute the backbone of the treatment strategy, and results from our and other centres suggest that monthly infusions of high-dose pulse i.v. methylprednisolone (HDPM) might stabilise the disease and hinder progression. This article provides an overview of the current evidence regarding treatment options for BO and presents the treatment results with HDPM in a paediatric national HSCT-cohort.
异基因造血干细胞移植(HSCT)后闭塞性细支气管炎(BO)是一种严重的并发症,影响 1.7%-26%的患者,报告死亡率为 21%-100%。它被认为是慢性移植物抗宿主病的表现,但我们对其病因和发病机制的了解仍然有限。正在制定诊断标准,这将使中心之间的研究活动更加统一和可比。目前,还没有完成可以证明有效治疗的随机对照试验。类固醇联合其他免疫抑制剂仍然是治疗策略的基础,我们和其他中心的结果表明,每月静脉注射大剂量脉冲甲基强的松龙(HDPM)可能稳定病情并阻止进展。本文综述了目前关于 BO 治疗选择的证据,并介绍了在儿科全国 HSCT 队列中使用 HDPM 的治疗结果。