骨髓增殖性疾病中的肺静脉闭塞病
Pulmonary veno-occlusive disease in myeloproliferative disorder.
作者信息
Willems E, Canivet J-L, Ghaye B, de Leval L, Radermecker M, Preiser J-C, Beguin Y
机构信息
Department of Medicine, Division of Haematology, University Hospital Centre, University of Liege, Domaine Universitaire du Sart-Tilman, Liege, Belgium.
出版信息
Eur Respir J. 2009 Jan;33(1):213-6. doi: 10.1183/09031936.00157707.
PMID:19118232
Abstract
The present study reports a case of biopsy-proven pulmonary veno-occlusive disease as a cause of severe pulmonary hypertension in a patient suffering from a chronic myeloproliferative disorder. The pulmonary disease evolved favourably under treatment with defibrotide, a pro-fibrinolytic medication used in hepatic veno-occlusive disease.
摘要
本研究报告了一例经活检证实的肺静脉闭塞性疾病，该疾病是一名患有慢性骨髓增殖性疾病患者严重肺动脉高压的病因。在使用去纤苷（一种用于治疗肝静脉闭塞性疾病的促纤溶药物）治疗后，肺部疾病病情好转。
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