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肝肺均受累的静脉闭塞性疾病。

Venoocclusive Disease With Both Hepatic and Pulmonary Involvement.

机构信息

Service de Pneumologie, Oncologie Thoracique et Allergologie Respiratoire, CHU de Besançon, Besançon, France.

Université; Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France; Service de Pneumologie, Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France; INSERM UMR-S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.

出版信息

Chest. 2020 Apr;157(4):e107-e109. doi: 10.1016/j.chest.2019.11.021.

Abstract

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.

摘要

肺静脉闭塞病(PVOD)是一种罕见的肺血管疾病,以肺动脉高压为特征,其特征是肺静脉系统优先受累。肝静脉闭塞病(HVOD),又称窦状隙梗阻综合征,是造血干细胞移植(HSCT)后 13%至 15%的患者中出现的一种病症。尽管肝静脉和肺静脉闭塞病可能具有一些共同的病理特征和一些病因,如 HSCT,但这两种疾病从未在单个成年患者中同时描述过。我们报告了一例接受 HSCT 并在 9 个月内发生 HVOD 和 PVOD 的患者。尽管存在差异,但 PVOD 和 HVOD 具有共同的危险因素和相关病症,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。

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