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

Swinkels, M.; Atiq, F.; Burgisser, P.E.; Moort, I. van; Meijer, K.; Eikenboom, J.; Fijnvandraat, K.; Galen, K.P.M. van; Meris, J. de; Schols, S.E.M.; Bom, J.G. van der; Cnossen, M.H.; Voorberg, J.; Leebeek, F.W.G.; Bierings, R.; Jansen, A.J.G.; WiN Study Grp

doi:doi:10.1111/bjh.18145

Swinkels, M.; Atiq, F.; Burgisser, P.E.; Moort, I. van; Meijer, K.; Eikenboom, J.; ... ; WiN Study Grp (2022)

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 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.