Marion Larue, Florent Malard, Ahmed S Alaskar, Mahmoud Aljurf, Mutlu Arat, Marie Balsat, Frédéric Baron, Grzegorz Basak, Ali Bazarbachi, Francesca Bonifazi, Eolia Brissot, Fabio Ciceri, Selim Corbacioglu, Fiona Dignan, Anne Huynh, Michelle Kenyon, Jürgen Kuball, Silvy Lachance, Tamas Masszi, Arnon Nagler, Shinichiro Okamoto, Antonio Pagliuca, Annalisa Ruggeri, Tapani Ruutu, Ibrahim Yakoub-Agha, Yishan Ye, Rafael F Duarte, Zinaida Perić, Enric Carreras, Mohamad Mohty
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引用次数: 0
摘要
窦状窦阻塞综合征(SOS)以前被称为静脉闭塞病(VOD)是一种潜在的致命并发症,主要发生在造血细胞移植后,特别是异体移植后。肝脏是受影响的主要器官,尽管其他器官,如肺,也可能在较小程度上受累。这种疾病的特点是肝小静脉梗阻,导致肝窦充血、肝缺血,严重者可导致暴发性肝衰竭。欧洲血液和骨髓移植学会(European Society for Blood and Marrow Transplantation)于2023年发布了最新的精细化诊断标准,提供了一种更准确的检测SOS/VOD的方法,可以根据疾病的严重程度进行早期干预,并更好地对患者进行分层。本文重点讨论肝脏SOS/VOD的主要危险因素,新的诊断方法和治疗策略,并强调早期使用去纤维肽,它仍然是严重SOS/VOD的参考治疗方法。
Management of liver sinusoidal obstruction syndrome/veno-occlusive disease in adults: a 2025 perspective from an international expert group.
Sinusoidal obstruction syndrome (SOS) formerly known as Veno-occlusive disease (VOD) is a potentially fatal complication that occurs mainly after haematopoietic cell transplantation, especially allogeneic transplantation. The liver is the principal organ affected, though other organs, such as the lungs, may also be involved to a lesser extent. The condition is characterised by obstruction of the hepatic venules, leading to sinusoidal congestion, hepatic ischaemia and, in severe cases, fulminant liver failure. Recent refined diagnostic criteria, published by the European Society for Blood and Marrow Transplantation in 2023, provide a more accurate method of detecting SOS/VOD, allowing earlier intervention and better stratification of patients according to the severity of their disease. This article focuses on liver SOS/VOD and discussing key risk factors, new diagnostic methods and therapeutic strategies, with an emphasis on the early use of defibrotide, which remains the reference treatment for severe SOS/VOD.
期刊介绍:
Bone Marrow Transplantation publishes high quality, peer reviewed original research that addresses all aspects of basic biology and clinical use of haemopoietic stem cell transplantation.
The broad scope of the journal thus encompasses topics such as stem cell biology, e.g., kinetics and cytokine control, transplantation immunology e.g., HLA and matching techniques, translational research, and clinical results of specific transplant protocols. Bone Marrow Transplantation publishes 24 issues a year.