Plasma levels of plasminogen activator inhibitor‐1 and bleeding phenotype in patients with von Willebrand disease

Introduction von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors,...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2017-05, Vol.23 (3), p.437-443
Main Authors: Abdul, S., Boender, J., Malfliet, J. J. M. C., Eikenboom, J., Fijn van Draat, K., Mauser‐Bunschoten, E. P., Meijer, K., Meris, J., Laros‐van Gorkom, B. A. P., Bom, J. G., Leebeek, F. W. G., Rijken, D. C., Uitte de Willige, S., Fijnvandraat, K, Coppens, M, Kors, A, Jonkers, H, Boersma, R, Beckers, A, Granzen, B, Tamminga, J, Linden, W, Ypma, F, Smiers, W, Brons, P, Nijziel, M, Cnossen, H
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Aged
Aged, 80 and over
Bleeding
bleeding score
Child
Child, Preschool
Cohort Studies
Female
Fibrinolysis
Genotype
Health risk assessment
Hemorrhage - complications
Humans
Infant
Male
Middle Aged
Phenotype
Plasma levels
Plasminogen Activator Inhibitor 1 - blood
Plasminogen Activator Inhibitor 1 - genetics
Plasminogen activator inhibitors
plasminogen activator inhibitor‐1
Population studies
von Willebrand disease
von Willebrand Diseases - blood
von Willebrand Diseases - complications
von Willebrand Diseases - genetics
Von Willebrand factor
Young Adult
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Summary:Introduction von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients. Aim To investigate whether plasminogen activator inhibitor‐1 (PAI‐1) level influences the variation in bleeding tendency in VWD patients. Methods PAI‐1 antigen levels were measured in the plasma of 633 patients with moderate or severe VWD who participated in the ‘Willebrand in the Netherlands’ (WiN) study, a nationwide multicentre cross‐sectional study. Bleeding severity was assessed using the Tosetto bleeding score. Results PAI‐1 levels increased with age (Spearman's rho: 0.225, P 
ISSN:1351-8216
1365-2516
DOI:10.1111/hae.13206