Budd-Chiari Syndrome in the Cirrhosis Stage Revealing Essential Thrombocythemia

A myeloproliferative syndrome, in particular an essential thrombocythemia (ET), is one of the most common causes (40-50%). The search for the Jak2 mutation facilitates haematological management. The diagnosis is made by Doppler ultrasound, supplemented by an MRI or CT scan. Support is based on early anti-coagulation, treatment of prothrombotic disease and complications of portal hypertension. Prognosis depends on the severity of the liver injury, the underlying prothrombotic condition and the hepatocellular carcinoma.