首页> 外文期刊>Biology of blood and marrow transplantation: journal of the American Society for Blood and Marrow Transplantation >Section XVII: Transplant Complications Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Novel Insights to Pathogenesis, Current Status of Treatment, and Future Directions
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Section XVII: Transplant Complications Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Novel Insights to Pathogenesis, Current Status of Treatment, and Future Directions

机译:第十七节:移植并发症造血干细胞移植后的肝静脉闭塞性疾病:发病机制,治疗现状和未来方向的新见解。

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INTRODUCTION Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, is a potentially life-threatening complication after hematopoietic stem cell transplantation (HSCT). VOD affects both adult and pediatric populations alike, with a higher incidence in the allogeneic setting . Disease onset is typically within the first 30 days after HSCT , although later occurrence has been reported . It is characterized by clinical features such as hepatomegaly, jaundice, weight gain, and ascites . VOD is reported to occur in 8% to 14% of patients after HSCT , although incidence rates may be as high as 60% in higher-risk patients (such as those with underlying liver disease and certain specific drug exposures, including gemtuzumab, ozogamicin, and sirolimus) and depending on the diagnostic criteria used . Severe VOD (sVOD) is typically associated with multiorgan failure (MOF) and high mortality rates (>80%) . Even among patients with moderate VOD, the mortality rate is still estimated at approximately 20% .
机译:引言肝静脉阻塞性疾病(VOD),也称为窦性阻塞综合征,是造血干细胞移植(HSCT)后可能危及生命的并发症。 VOD会影响成人和儿童人群,在同种异体环境中发病率更高。疾病发作通常发生在HSCT后的前30天内,尽管有报道后来发生。它的特征是临床特征,例如肝肿大,黄疸,体重增加和腹水。据报道,HSCT后VOD发生在8%至14%的患者中,尽管高危患者的发病率可能高达60%(例如患有基础肝病和某些特定药物暴露的患者,包括吉妥珠单抗,ozogamicin,和西罗莫司),并取决于所用的诊断标准。严重的VOD(sVOD)通常与多器官衰竭(MOF)和高死亡率(> 80%)有关。即使在具有中等VOD的患者中,死亡率仍然估计约为20%。

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