Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria

M F McMullin, P Hillmen, J Jackson, P Ganly, L Luzzatto

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60 Citations (Scopus)

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.
Original languageEnglish
Pages (from-to)85-9
Number of pages5
JournalEuropean Journal of Internal Medicine
Volume235
Issue number1
Publication statusPublished - Jan 1994

Keywords

  • Adult
  • Budd-Chiari Syndrome
  • Female
  • Hemoglobinuria, Paroxysmal
  • Humans
  • Tissue Plasminogen Activator

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