4.6 Article

Pulmonary veno-occlusive disease in myeloproliferative disorder

Journal

EUROPEAN RESPIRATORY JOURNAL
Volume 33, Issue 1, Pages 213-216

Publisher

EUROPEAN RESPIRATORY SOC JOURNALS LTD
DOI: 10.1183/09031936.00157707

Keywords

Anagrelide; chronic myeloproliferative disorder; defibrotide; pulmonary hypertension; pulmonary veno-occlusive disease

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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.

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