Abstract
The Budd-Chiari syndrome (BCS) is an uncommon and life-threatening disorder defined as the obstruction of hepatic venous outflow regardless of its causative mechanism or level of obstruction. The clinical presentation of BCS is highly variable and can range from asymptomatic cases to fulminant hepatic failure with encephalopathy. In the vast majority of cases, it is possible to identify an inherited or acquired prothrombotic risk factor as the underlying cause of thrombosis being chronic myeloproliferative diseases the most frequent etiological cause of BCS. In many cases, multiple factors are present. Anticoagulation is mandatory in patients with BCS. The need for an additional intervention, such as hepatic vein angioplasty, thrombolysis, transjugular intrahepatic portosystemic shunt, surgical shunts, or liver transplantation, depends on the severity of symptoms and response to treatment.KeywordsChronic myeloproliferative disease JAK2V617F mutationProthrombotic disorderAnticoagulationSurgical shuntTransjugular intrahepatic portosystemic shuntLiver transplantation
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