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Pulmonary veno-occlusive disease in myeloproliferative disorder

¹ Dept of Medicine, Division of Haematology, and Depts of ² General Intensive Care, ³ Radiology, ⁴ Pathology, and ⁵ Cardiothoracic Surgery, University Hospital Centre, University of Liege, Domaine Universitaire du Sart-Tilman, Liege, Belgium.

CORRESPONDENCE: J-L. Canivet, Dept of General Intensive Care, University Hospital Centre, Domaine Universitaire du Sart-Tilman, B-4000 Liege, Belgium. Fax: 32 43668898. E-mail: Jean-Luc.Canivet{at}chu.ulg.ac.be

Keywords: Anagrelide, chronic myeloproliferative disorder, defibrotide, pulmonary hypertension, pulmonary veno-occlusive disease

Received: November 23, 2007
Accepted August 19, 2008

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.