title: Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria.
abstract: Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder thought to arise in a multipotent haemopoietic stem cell. A distinct clinical feature is a tendency to thrombosis, with a particular predilection for the hepatic veins (Budd-Chiari syndrome). We report here on two patients with PNH who developed hepatic vein thrombosis (HVT) and who were treated with tissue plasminogen activator (t-PA). Both patients had a marked clinical and radiological improvement following the t-PA treatment and remain well over 2 years and 6 years after the treatment. This method of thrombolysis for HVT occurring in PNH has only been reported in two previous patients with limited follow-up. We suggest that this therapy is a useful first-line treatment for PNH patients who develop HVT.
authors.affiliation: Department of Haematology, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK.
authors.forename: M F
authors.initials: MF
authors.lastname: McMullin
authors.forename: P
authors.initials: P
authors.lastname: Hillmen
authors.forename: J
authors.initials: J
authors.lastname: Jackson
authors.forename: P
authors.initials: P
authors.lastname: Ganly
authors.forename: L
authors.initials: L
authors.lastname: Luzzatto
mesh_terms: D000328:Adult; D006502:Budd-Chiari Syndrome / Q000188:drug therapy* / Q000209:etiology; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications*; D006801:Humans; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use*
chemical_list: D010959:Tissue Plasminogen Activator