Platelet degranulation and bleeding phenotype in a large cohort of Von Willebrand disease patients
WiN Study Group
(2022) British Journal of Haematology, volume 197, issue 4, pp. 497 - 501
(Article)
Abstract
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)
... read more
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.
show less
Download/Full Text
Keywords: bleeding disorders, platelet activation, platelet factor 4, VWD, VWF, Hemorrhage/etiology, Phenotype, Humans, von Willebrand Factor/genetics, Platelet Factor 4, von Willebrand Diseases/genetics, Hematology, Research Support, Non-U.S. Gov't, Journal Article
ISSN: 0007-1048
Publisher: Wiley-Blackwell
Note: Funding Information: F. W. G. Leebeek received research support from CSL Behring and Shire for performing the Willebrand in the Netherlands (WiN) study, and is consultant for uniQure, Biomarin, Novo Nordisk and Shire, of which the fees go to the institution. F. Atiq received the CSL Behring‐Heimburger Award 2018, and a travel grant from Sobi. I. van Moort received the CSL Behring‐Heimburger Award 2021. A. J. G. Jansen received speaker fees and travel cost payments from 3SBio, Amgen and Novartis, is on the international advisory board at Novartis and received research support from Sanofi, Argenx and CSL Behring. J. Eikenboom received research support from CSL Behring and he has been a teacher on educational activities of Roche. K. P. M. van Galen received unrestricted research support from CSL Behring and Bayer. J. G. van der Bom has received unrestricted research/educational funding for various projects from the following companies: Bayer Schering Pharma, Baxter, CSL Behring, Novo Nordisk, and Pfizer. In addition, she has been a consultant to Baxter and Pfizer, and she has been a teacher on educational activities of Bayer Schering Pharma. M. H. Cnossen has received unrestricted research/educational and travel funding from the following companies: Pfizer, Baxter, Bayer Schering Pharma, CSL Behring, Novo Nordisk and Novartis, and serves as a member on steering boards of Roche and Bayer of which fees go to the institution. K. Fijnvandraat is a member of the European Haemophilia Treatment and Standardization Board sponsored by Baxter, has received unrestricted research grants from CSL Behring and Bayer, and has given lectures at educational symposiums organized by Pfizer, Bayer and Baxter. K. Meijer received speaker fees from Alexion, Bayer and CSL Behring, fees for participation in trial steering committee for Bayer, consulting fees from Uniqure, and fees for participation in data monitoring and endpoint adjudication committee for Octapharma. S. Schols received travel grants from Bayer and Takeda and consultancy grants from Takeda and Novo Nordisk. None of the other authors has a conflict of interest to declare. Funding Information: This work was supported by grants from the Landsteiner Stichting voor Bloedtransfusie Research (LSBR-1707) (Ruben Bierings), the Netherlands Organization for Scientific Research (NWO NWA.1160.18.038) (Ruben Bierings and Iris van Moort) and an EHA Clinical Research Fellowship (A. J. Gerard Jansen). The WiN study was supported (in part) by research funding from the Dutch Haemophilia Foundation (Stichting Haemophilia) (Frank W. G. Leebeek) and CSL Behring (Frank W. G. Leebeek, unrestricted grant). Funding Information: This work was supported by grants from the Landsteiner Stichting voor Bloedtransfusie Research (LSBR‐1707) (Ruben Bierings), the Netherlands Organization for Scientific Research (NWO NWA.1160.18.038) (Ruben Bierings and Iris van Moort) and an EHA Clinical Research Fellowship (A. J. Gerard Jansen). The WiN study was supported (in part) by research funding from the Dutch Haemophilia Foundation (Stichting Haemophilia) (Frank W. G. Leebeek) and CSL Behring (Frank W. G. Leebeek, unrestricted grant). Publisher Copyright: © 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.
(Peer reviewed)
