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造血细胞移植后发生肝静脉闭塞病/窦状隙阻塞综合征且需要呼吸机或透析依赖的患者,在第 100 天defibrotide 治疗的生存汇总分析。

Pooled analysis of Day 100 survival for defibrotide-treated patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome and ventilator or dialysis dependence following haematopoietic cell transplantation.

机构信息

Division of Hematologic Malignancy, Department of Medical Oncology, Jerome Lipper Multiple Myeloma Center, Harvard Medical School, Dana-Farber Cancer Institute, Boston, MA, USA.

Division of Pediatric Blood and Marrow Transplantation, University of Minnesota, Minneapolis, MN, USA.

出版信息

Br J Haematol. 2020 Aug;190(4):583-587. doi: 10.1111/bjh.16552. Epub 2020 Mar 10.

DOI:10.1111/bjh.16552
PMID:32157682
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7497046/
Abstract

For patients with untreated hepatic veno-occlusive disease (VOD)/sinusoidal obstruction syndrome (SOS) with multi-organ dysfunction (MOD), mortality is >80%. We conducted a pooled analysis of three studies that assessed Day 100 survival in relationship to MOD severity, with dialysis and/or ventilator dependence representing the most severe organ dysfunction. All patients in the analysis were diagnosed using Baltimore criteria/biopsy. This analysis of patients with VOD/SOS and MOD after haematopoietic cell transplantation (HCT; n = 651) demonstrated higher Day 100 survival rates amongst defibrotide-treated patients with VOD/SOS with less versus more severe forms of MOD. Even patients with severe forms of MOD post-HCT benefitted from defibrotide.

摘要

对于未经治疗的肝静脉闭塞病(VOD)/窦状隙阻塞综合征(SOS)伴多器官功能障碍(MOD)的患者,死亡率>80%。我们对三项研究进行了汇总分析,这些研究评估了 100 天存活率与 MOD 严重程度的关系,其中透析和/或呼吸机依赖代表最严重的器官功能障碍。所有分析中的患者均根据巴尔的摩标准/活检进行诊断。这项对造血细胞移植(HCT)后发生 VOD/SOS 和 MOD 的患者的分析表明,与 MOD 较轻的患者相比,接受治疗的 VOD/SOS 患者的 100 天存活率更高。即使是在 HCT 后出现严重 MOD 的患者,也能从地塞米松治疗中获益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f01d/7497046/d73b7ebf3770/BJH-190-583-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f01d/7497046/d73b7ebf3770/BJH-190-583-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f01d/7497046/d73b7ebf3770/BJH-190-583-g001.jpg

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