Pulmonary veno-occlusive disease in myeloproliferative disorder

E Willems; J-L Canivet; B Ghaye; L de Leval; M Radermecker; J-C Preiser; Y Beguin

doi:10.1183/09031936.00157707

Pulmonary veno-occlusive disease in myeloproliferative disorder

Eur Respir J. 2009 Jan;33(1):213-6. doi: 10.1183/09031936.00157707.

Authors

E Willems¹, J-L Canivet, B Ghaye, L de Leval, M Radermecker, J-C Preiser, Y Beguin

Affiliation

¹ Department of Medicine, Division of Haematology, University Hospital Centre, University of Liege, Domaine Universitaire du Sart-Tilman, Liege, Belgium.

PMID: 19118232
DOI: 10.1183/09031936.00157707

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.

Publication types

Case Reports

MeSH terms

Aged
Female
Fibrinolytic Agents / therapeutic use
Humans
Myeloproliferative Disorders / complications*
Myeloproliferative Disorders / pathology
Polydeoxyribonucleotides / therapeutic use
Pulmonary Veno-Occlusive Disease / diagnosis*
Pulmonary Veno-Occlusive Disease / drug therapy
Pulmonary Veno-Occlusive Disease / etiology*

Substances

Fibrinolytic Agents
Polydeoxyribonucleotides
defibrotide