Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome

Jildou Hoekstra, Ana H C Guimarães, Frank W G Leebeek, Sarwa Darwish Murad, Joyce J M C Malfliet, Aurelie Plessier, Manuel Hernandez-Guerra, Philippe Langlet, Elwyn Elias, Jonel Trebicka, Massimo Primignani, Juan Carlos Garcia-Pagan, Dominique C. Valla, Dingeman C. Rijken, Harry L A Janssen

Research output: Contribution to journalArticlepeer-review


In Budd-Chiari syndrome (BCS), thrombosis develops in the hepatic veins or inferior vena cava. To study the relationship between hypofibrinolysis and BCS, we measured plasma levels of fibrinolysis proteins in 101 BCS patients and 101 healthy controls and performed a plasmabased clot lysis assay. In BCS patients, plasminogen activator inhibitor 1 (PAI-1) levels were significantly higher than in controls (median, 6.3 vs 1.4 IU/mL,. P <.001). Thrombin-activatable fibrinolysis inhibitor and plasmin inhibitor levels were lower than in controls (13.8 vs 16.9 μg/mL and 0.91 vs 1.02 U/L, both P <.001). Median plasma clot lysis time (CLT) was 73.9 minutes in cases and 73.0 minutes in controls (P = .329).Asubgroup of cases displayed clearly elevated CLTs. ACLT above the 90th or 95th percentile of controls was associated with an increased risk of BCS, with odds ratios of 2.4 (95% confidence interval, 1.1-5.5) and 3.4 (95% confidence interval, 1.2-9.7), respectively. In controls, only PAI-1 activity was significantly associated with CLT. Analysis of single nucleotide polymorphisms of fibrinolysis proteins revealed no significant differences between cases and controls. This case-control study provides the first evidence that an impaired fibrinolytic potential, at least partially caused by elevated PAI-1 levels, is related to the presence of BCS.

Original languageEnglish
Pages (from-to)388-395
Number of pages8
Issue number2
Publication statusPublished - Jan 14 2010

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology


Dive into the research topics of 'Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome'. Together they form a unique fingerprint.

Cite this