Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients

Maurice Swinkels, Nasirah Atiq, WiN study group, Petra Bürgisser, Iris van Moort, Karina Meijer, Jeroen C.J. Eikenboom, Karin Fijnvandraat, Karin P.M. van Galen, Joke De Meris, Saskia E.M. Schols, Johanna G. van der Bom, Marjon Cnossen, Jan Voorberg, Frank Leebeek, Ruben Bierings, Gerard Jansen*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


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.
Original languageEnglish
Pages (from-to)497-501
Number of pages5
JournalBritish Journal of Haematology
Issue number4
Early online date22 Mar 2022
Publication statusPublished - May 2022

Bibliographical note

This work was supported by grants from the Landsteiner Stichting voor Bloedtransfusie Research (LSBR-1707), the Netherlands Organization for Scientific Research (NWO NWA.1160.18.038) and an EHA Clinical Research Fellowship. The WiN study was supported (in part) by research funding from the Dutch Haemophilia Foundation (Stichting Haemophilia) and CSL Behring (unrestricted grant).


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