Abstract
Polycythemia vera-induced Budd-Chiari syndrome in an older patient The case of a 94-year-old patient with subacute Budd-Chiari syndrome (BCS) caused by a novel diagnosis of polycythemia vera (PV) is reported. BCS is mostly seen in young or middle-aged patients. The presentation in a nonagenarian is rare, making this case exceptional. BCS is defined by a hepatic venous outflow obstruction. Its clinical presentation is variable from fulminant liver failure to an insidious form with symptoms of cirrhosis at the time of the diagnosis. In western countries, primary BCS is mainly seen, which is caused by an endoluminal lesion. A hypercoagulable state provoked by myeloproliferative neoplasms (MPN) is mostly responsible. The patient presented with abdominal distention and anorexia since two months. Physical examination revealed hepatomegaly and ascites. Laboratory data indicated polycythemia and cholestasis. The CT scan of the abdomen was diagnostic for subacute BCS. A JAK2-V617F mutation was found. The therapy consisted of anticoagulation, low-dose acetylsalicylic acid, phlebotomies and supportive care with diuretics and paracentesis.
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