Polycythemia Vera Presenting with Fulminant Hepatic Failure Due to Acute Budd-Chiari Syndrome: Case Report

We describe a 38 year-old woman with polycythemia vera who presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. She had a history of abdominal pain and distention for 4 days. Laboratory and clinical findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and doppler ultrasonography showing ascites, hepatomegaly, portal hypertension and total occlusion of hepatic veins. Complete blood count and other clinical findings were compatible with polycythemia vera. The patient was successfully treated with urgent administration of continuous heparin infusion, repeated phlebotomies and hydroxyurea. We emphasize that early diagnosis and effective treatment in such fulminant cases can be life saving.