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

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 platelet...

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Detalles Bibliográficos
Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Platelets, Thrombosis and Haemostasis
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9314899/
https://www.ncbi.nlm.nih.gov/pubmed/36165954
http://dx.doi.org/10.1111/bjh.18145
Descripción
Sumario: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.

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