Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation.

Diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation.
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DOI:
10.1038/bmt.2017.161
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发表时间:
2018-03
影响因子:
4.8
通讯作者:
Bader P
Bader P
中科院分区:
医学3区
文献类型:
--
作者:
Corbacioglu S;Carreras E;Ansari M;Balduzzi A;Cesaro S;Dalle JH;Dignan F;Gibson B;Guengoer T;Gruhn B;Lankester A;Locatelli F;Pagliuca A;Peters C;Richardson PG;Schulz AS;Sedlacek P;Stein J;Sykora KW;Toporski J;Trigoso E;Vetteranta K;Wachowiak J;Wallhult E;Wynn R;Yaniv I;Yesilipek A;Mohty M;Bader P

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在过去的十年中,造血细胞移植(HCT)的进展已经导致移植相关死亡率低于15%。肝窦阻塞综合征/静脉闭塞性疾病(SOS/VOD)是HCT的一种危及生命的并发症,属于一组越来越被认为是与移植相关的全身性内皮疾病的疾病。在大多数情况下,SOS/VOD在几周内解决;然而,严重的SOS/VOD导致多器官功能障碍/衰竭,死亡率约为80%。及时诊断SOS/VOD是至关重要的,考虑到可获得的治疗方案具有良好的耐受性。目前的诊断标准适用于成人和儿童。然而,在过去的十年中,SOS/VOD在发病率,遗传易感性,临床表现,预防,治疗和结果方面在年龄组之间存在显着差异。提高对SOS/VOD的理解和有效治疗的可用性对巴尔的摩和西雅图诊断儿童SOS/VOD标准的使用提出了质疑。本立场文件的目的是代表欧洲血液和骨髓移植协会提出儿童SOS/VOD的新诊断和严重程度标准。
The advances in hematopoietic cell transplantation (HCT) over the last decade have led to a transplant-related mortality below 15%. Hepatic sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication of HCT that belongs to a group of diseases increasingly identified as transplant-related, systemic endothelial diseases. In most cases, SOS/VOD resolves within weeks; however, severe SOS/VOD results in multi-organ dysfunction/failure with a mortality rate >80%. A timely diagnosis of SOS/VOD is of critical importance, given the availability of therapeutic options with favorable tolerability. Current diagnostic criteria are used for adults and children. However, over the last decade it has become clear that SOS/VOD is significantly different between the age groups in terms of incidence, genetic predisposition, clinical presentation, prevention, treatment and outcome. Improved understanding of SOS/VOD and the availability of effective treatment questions the use of the Baltimore and Seattle criteria for diagnosing SOS/VOD in children. The aim of this position paper is to propose new diagnostic and severity criteria for SOS/VOD in children on behalf of the European Society for Blood and Marrow Transplantation.
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