Buddův-Chiariho syndrom u pacientky s primárnítrombocytémií léčené interferonem alfa řešenýtransjugulární portosystémovou spojkou

Budd-Chiari Syndrome in Patient with Primary ThrombocythaemiaTreated with Interferon Alpha Medically Managed by Transjugular Porto-systemic Shunt

The case report of a young female patient with personal history of primary thrombocythaemia, treated with interferonalpha, admitted to our medical department for severe abdominal pain, hepatomegaly, ascites and alteration of hepaticfunction is presented. Magnetic resonance imaging showed the picture typical for Budd-Chiari syndrome caused byexternal obstruction of the intrahepatal portion of inferior vena cava. The cause of the syndrome remains uncertain,possibility of the haematogenic infiltration of the liver or venal thrombosis within primary or secondary (interferon- induced) antiphospholipid syndrome is discussed. Liver biopsy could elucidate the exact cause, but it was notperformed for technical problems.

Key words:
Budd-Chiari syndrome, primary thrombocythaemia, interferon alpha, antiphospholipid syndrome,infiltration of organs, TIPS.