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.
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.
Breccia
Massimo
M
Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy. breccia@bce.uniroma1.it
Morano
Salvatore Giacomo
SG
D'Andrea
Mariella
M
Russo
Eleonora
E
D'Elia
Gianna Maria
GM
Alimena
Giuliana
G
D000328:Adult; D025861:Blood Coagulation Disorders, Inherited / Q000150:complications / Q000235:genetics / Q000628:therapy; D006502:Budd-Chiari Syndrome / Q000209:etiology* / Q000235:genetics / Q000628:therapy; D005192:Family Health; D005260:Female; D006801:Humans; D009154:Mutation; D011087:Polycythemia Vera / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D020151:Protein C Deficiency; D018455:Protein S Deficiency; D011516:Prothrombin; D019851:Thrombophilia / Q000150:complications* / Q000235:genetics / Q000628:therapy
D011516:Prothrombin