Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients. (22nd March 2022)

• Record Type: Journal Article
• Authors: Swinkels, Maurice; Atiq, Ferdows; Bürgisser, Petra E.; van Moort, Iris; Meijer, Karina; Eikenboom, Jeroen; Fijnvandraat, Karin; van Galen, Karin P. M.; de Meris, Joke; Schols, Saskia E. M.; van der Bom, Johanna G.; Cnossen, Marjon H.; Voorberg, Jan; Leebeek, Frank W. G.; Bierings, Ruben; Jansen, A. J. Gerard
• Other Names: Fijnvandraat K. investigator.; Coppens M. investigator.; de Meris J. investigator.; Nieuwenhuizen L. investigator.; Meijer K. investigator.; Tamminga R. Y. J. investigator.; Ypma P. F. investigator.; Eikenboom H. C. J. investigator.; van der Bom J. G. investigator.; Smiers F. J. W. investigator.; Granzen B. investigator.; Moenen F. investigator.; Brons P. investigator.; Schols S. E. M. investigator.; Leebeek F. W. G. investigator.; Cnossen M. H. investigator.; Atiq F. investigator.; van Kwawegen C. B. investigator.; van Galen K. P. M. investigator.
• Is Part Of: British journal of haematology. Volume 197:Number 4(2022)
• Page Start: 497
• Page End: 501
• Publication Date: 2022-03-22
• Subjects: bleeding disorders -- platelet activation -- platelet factor 4 -- VWD -- VWF; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.18145
• Languages: English
• ISSNs: 0007-1048

Abstract

Summary: Von Willebrand disease (VWD) is a bleeding disorder caused by quantitative (type 1 or 3) or qualitative (type 2A/2B/2M/2N) defects of circulating von Willebrand factor (VWF). Circulating VWF levels not always fully explain bleeding phenotypes, suggesting a role for alternative factors, like platelets. Here, we investigated platelet factor 4 (PF4) in a large cohort of patients with VWD. PF4 levels were lower in type 2B and current bleeding phenotype was significantly associated with higher PF4 levels, particularly in type 1 VWD. Based on our findings we speculate that platelet degranulation and cargo release may play a role across VWD subtypes.