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Venoocclusive Disease With Both Hepatic and Pulmonary Involvement
Chest ( IF 9.5 ) Pub Date : 2020-04-01 , DOI: 10.1016/j.chest.2019.11.021
Noémie Tissot , David Montani , Marie-France Seronde , Bruno Degano , Thibaud Soumagne

Pulmonary venoocclusive disease (PVOD) is a rare form of pulmonary vascular disease with pulmonary hypertension characterized by preferential involvement of the pulmonary venous system. Hepatic venoocclusive disease (HVOD), also known as sinusoidal obstruction syndrome, is a condition that occurs in 13% to 15% of patients after hematopoietic stem cell transplantation (HSCT). Although hepatic and pulmonary venoocclusive diseases may share some pathologic features as well as some etiologies such as HSCT, these two disorders have never been described together in a single adult patient. We report the case of a patient who received HSCT and developed HVOD and PVOD within 9 months. Despite their differences, PVOD and HVOD share common risk factors and associated conditions, suggesting that in the context of HSCT, the two diseases share common pathophysiological mechanisms. Optimal treatment for HSCT-related PVOD remains to be determined.

中文翻译:

伴有肝肺受累的静脉闭塞性疾病

肺静脉闭塞性疾病 (PVOD) 是一种罕见的肺血管疾病,以肺静脉系统优先受累为特征的肺动脉高压。肝静脉闭塞性疾病 (HVOD),也称为肝窦阻塞综合征,是一种在 13% 至 15% 的造血干细胞移植 (HSCT) 后患者中发生的疾病。尽管肝和肺静脉闭塞性疾病可能具有一些病理特征以及一些病因,例如造血干细胞移植,但这两种疾病从未在一个成年患者中一起被描述过。我们报告了一名接受 HSCT 并在 9 个月内发展为 HVOD 和 PVOD 的患者的病例。尽管存在差异,PVOD 和 HVOD 具有共同的风险因素和相关条件,这表明在 HSCT 的背景下,这两种疾病具有共同的病理生理机制。HSCT 相关 PVOD 的最佳治疗方法仍有待确定。
更新日期:2020-04-01
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