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Budd‐Chiari syndrome as the first manifestation of polycythemia vera in young women with inherited thrombophilic state: an aggressive form of myeloproliferative disorder requiring multidisciplinary management
Author(s) -
Breccia Massimo,
Morano Salvatore Giacomo,
D'Andrea Mariella,
Russo Eleonora,
D'Elia Gianna Maria,
Alimena Giuliana
Publication year - 2005
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2005.00522.x
Subject(s) - polycythemia vera , medicine , budd–chiari syndrome , concomitant , myeloproliferative disorders , intervention (counseling) , complication , pediatrics , thrombophilia , surgery , thrombosis , psychiatry , inferior vena cava
The Budd‐Chiari syndrome (BCS), characterized by the obstruction and occlusion of the suprahepatic veins, is a rare but typical complication occurring in patients with polycythemia vera (PV). We describe three young women who developed BCS as first manifestation of PV, in association with an inherited thrombophilic state and in the absence of concomitant use of oral contraceptives. Our report illustrates the existence of an aggressive form of myeloproliferative disorder, which requires prompt recognition and immediate therapeutic intervention including cytostatic drugs and anticoagulant treatment. Furthermore, we suggest the need of routine screening for thrombophilic state in young women affected by PV.