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The defective down regulation of fibrinolysis in haemophilia a can be restored by increasing the tafi plasma concentration

Academic Article
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Overview

authors

  • Mosnier, Laurent
  • Lisman, T.
  • van den Berg, H. M.
  • Nieuwenhuis, H. K.
  • Meijers, J. C. M.
  • Bouma, B. N.

publication date

  • October 2001

journal

  • Thrombosis and Haemostasis  Journal

abstract

  • TAFI (thrombin activatable fibrinolysis inhibitor) down regulates fibrinolysis after activation by relatively high concentrations of thrombin generated during coagulation via thrombin mediated factor XI activation and subsequent activation of the intrinsic pathway. It is this secondary burst of thrombin that is severely diminished in haemophilia A, a deficiency of coagulation factor VIII. We therefore investigated the role of TAFI in haemophilia A by measuring the clot lysis times of tissue factor induced fibrin formation and tPA mediated fibrinolysis. In haemophilia A plasma clot lysis times were normal at relatively high tissue factor concentrations but severely decreased at moderate to low tissue factor concentrations, indicating that the thrombin generation via the extrinsic pathway was insufficient to activate TAFI. Addition of factor VIII, TAFI or thrombomodulin restored the clot lysis times at low tissue factor concentrations. This confirms the hypothesis that the bleeding disorder in haemophilia A is not merely a defect in the initial clot formation but is in fact a triple defect: reduced thrombin formation via the extrinsic pathway at low tissue factor concentrations, a reduced secondary burst of thrombin generation via the intrinsic pathway and a defective down regulation of the fibrinolytic system by the intrinsic pathway.

subject areas

  • Adolescent
  • Adult
  • Biomarkers
  • Blood Coagulation
  • Blood Coagulation Tests
  • Carboxypeptidase B2
  • Child
  • Factor VIII
  • Fibrinolysis
  • Hemophilia A
  • Humans
  • Male
  • Peptide Fragments
  • Prothrombin
  • Thrombin
  • Thrombomodulin
  • Thromboplastin
  • Time Factors
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Research

keywords

  • carboxypeptidase U
  • coagulation
  • factor VIII
  • fibrinolysis
  • haemophilia A
  • thrombin activatable fibrinolysis inhibitor (TAFI)
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Identity

International Standard Serial Number (ISSN)

  • 0340-6245

PubMed ID

  • 11686321
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Additional Document Info

start page

  • 1035

end page

  • 1039

volume

  • 86

issue

  • 4

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