Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome.

Paroxysmal nocturnal hemoglobinuria (PNH) is associated with a high risk of thrombosis, particularly in the peripheral, cerebral, and abdominal veins. We report a patient with an occlusion of the hepatic veins and a slit shape narrowing of the cava inferior consistent with the Budd-Chiari syndrome in whom intravenous fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was applied. Systemic rt-PA was given in a dose of 25 mg rt-PA over 3 h and 25 mg rt-PA as constant intravenous infusion over the next 21 h leading to an incomplete recanalization. The same protocol was applied again 2 days later, resulting in a complete recanalization of the hepatic veins and the vena cava inferior. Our case shows that exclusive systemic application of rt-PA can result in full anatomic and clinical restoration.