title: Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome.
abstract: 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.
authors.affiliation: Department of Internal Medicine III, Division of Nephrology and Dialysis, University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria. anna-christine.hauser@univie.ac.at
authors.forename: A C
authors.initials: AC
authors.lastname: Hauser
authors.forename: A
authors.initials: A
authors.lastname: Brichta
authors.forename: I
authors.initials: I
authors.lastname: Pabinger-Fasching
authors.forename: U
authors.initials: U
authors.lastname: Jäger
mesh_terms: D000328:Adult; D001835:Body Weight / Q000187:drug effects; D006502:Budd-Chiari Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007262:Infusions, Intravenous; D015912:Thrombolytic Therapy / Q000379:methods*; D010959:Tissue Plasminogen Activator / Q000008:administration & dosage*; D016896:Treatment Outcome; D014463:Ultrasonography
chemical_list: D010959:Tissue Plasminogen Activator