Fulminant Budd-Chiari syndrome associated with polycythemia rubra Vera and factor V Leiden mutation
Makaleler > Tam Makale · 2006
Yazarlar: Murat AKYILDIZ, Abdullah Zeki KARASU, Hamad Dheir, Necla Osmanoglu, Sinan Akay, Tankut Ilter
EUROPEAN JOURNAL OF INTERNAL MEDICINE, SCI Expanded İndekslerine Giren Dergi, Uluslararası, 2006, cilt 17, ss 66-67
DOI: 10.1016/j.ejim.2005.08.007
Özet
Budd-Chiari syndrome (BCS) is a severe disorder characterized by hepatic venous outflow obstruction. Hypercoagulable states are the major etiological factors for the development of BCS and can be identified in about 75% of patients. Multiple etiological factors can be found in the same patient. Hematologic abnormalities, especially myeloproliferative disorders, are the most common causes of BCS. Furthermore, the prevalence of factor V Leiden mutation is three times greater in patients with BCS. Although the clinical course tends to be chronic, BCS may, on rare occasions, cause acute liver failure. Herein, we report a patient who had factor V Leiden mutation and polycythemia rubra vera, presented as fulminant BCS. (C) 2005 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.