Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission

L. A. Lotta, R. Lombardi, M. Mariani, S. Lancellotti, R. De Cristofaro, M. J. Hollestelle, M. T. Canciani, P. M. Mannucci, F. Peyvandi

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Binding of von Willebrand factor (VWF) multimers of ultra-large size to platelets is considered the triggering mechanism of microvascular thrombosis in thrombotic thrombocytopenic purpura (TTP). Objective: To assess the potential of VWF-related measurements as markers of disease activity and severity in TTP. Methods: VWF antigen (VWF:Ag), platelet glycoprotein-Ib-α binding-conformation (GPIb-α/BC) and multimeric pattern were investigated in 74 patients with acquired TTP during acute disease, remission or both and 73 healthy controls. In patients with both acute and remission samples available, VWF ristocetin co-factor activity (VWF:RCo) and collagen binding (VWF:CB) were also measured. The relationships of study measurements with the presence of acute disease and remission and with markers of disease severity were assessed. Results: VWF:Ag and VWF-GPIb-α/BC were higher in TTP patients than controls (P

Original languageEnglish
Pages (from-to)1744-1751
Number of pages8
JournalJournal of Thrombosis and Haemostasis
Volume9
Issue number9
DOIs
Publication statusPublished - Sep 2011

Keywords

  • ADAMTS-13
  • Multimers
  • Platelet reactive von Willebrand factor
  • Thrombotic thrombocytopenic purpura
  • Von Willebrand factor

ASJC Scopus subject areas

  • Hematology

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