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Polycythemia Vera Presenting with Fulminant Hepatic Failure Due to ...

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Topic: Polycythemia Vera
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Polycythemia Vera Presenting with Fulminant Hepatic Failure Due to Acute Budd-Chiari Syndrome: Case Report INTRODUCTION Budd-Chiari syndrome (BCS) is a rare syndrome resulting from obstruction of hepatic vein or inferior vena cava and presenting with painful hepatomegaly and ascites. Clinical presentation varies depending on the time and degree of the obstruction 1,2 . If the occlusion is total and rapid, the patient may present with fulminant hepatic failure. Mostly encountered causes of BCS are myeloproliferative diseases, especially polycythemia vera (PV), paroxysmal nocturnal hemoglobinuria, and other hypercoagulability states 4-11 . Although the prognosis of acute BCS is poor, with early diagnosis and effective treatment, the outcome may be favorable. The case described here, is an acute form of BCS presented with fulminant hepatic fa43 Polycythemia Vera Presenting with Fulminant Hepatic Failure Due to Acute Budd-Chiari Syndrome: Case Report Sleyman Sami KARTI*, Mustafa YILMAZ*, Elif ALTUN**, Polat KOUCU***, Mehmet ARSLAN****, Ercment OVALI* * Department of Hematology, School of Medicine, Karadeniz Technical University ** Department of Internal Medicine, School of Medicine, Karadeniz Technical University *** Department of Radiology, School of Medicine, Karadeniz Technical University **** Department of Gastroenterology, School of Medicine, Karadeniz Technical University, Trabzon, TURKEY ABSTRACT 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 ...

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