An update on the von Willebrand factor collagen binding assay: 21 years of age and beyond adolescence but not yet a mature adult

Semin Thromb Hemost. 2007 Nov;33(8):727-44. doi: 10.1055/s-2007-1000364.

Abstract

von Willebrand disease (VWD) is considered to be the most common inherited bleeding disorder. It is diagnosed after a clinical and physical review, with personal and familial evidence of (primarily mucocutaneous) bleeding, and confirmed by laboratory testing. The latter typically entails initial plasma testing of factor VIII coagulant (FVIII:C), von Willebrand factor (VWF) protein (antigen; VWF:Ag), and VWF function, which has classically been assessed using the ristocetin cofactor (VWF:RCo) assay. More recent attention has focused on another functional VWF assay, the collagen binding (VWF:CB) assay, as a possible replacement for the VWF:RCo assay or as a supplementary test of VWF adhesive "activity." Additional laboratory testing can comprise a battery of confirmatory and VWD subtype assisting assays, including assessment of VWF:multimers. This review updates our knowledge of VWD diagnostics with a particular emphasis on the VWF:CB assay. There is good evidence now in place that an optimized VWF:CB assay can significantly reduce the diagnostic error rate otherwise arising from the use of a test panel restricted to including the VWF:RCo assay as the sole functional VWF assay. Nevertheless, the VWF:CB assay should not be used to wholly replace the VWF:RCo assay in phenotypic testing but rather as a supplementary assay. However, with some thought and justification, the VWF:CB assay can be used to partly replace the VWF:RCo assay in some "screening" applications and can also be used to abrogate the need to perform routine VWF:multimers in most test cases.

Publication types

  • Review

MeSH terms

  • Collagen / metabolism*
  • Diagnostic Errors
  • Humans
  • Ristocetin / metabolism
  • von Willebrand Diseases / diagnosis*
  • von Willebrand Factor / analysis*
  • von Willebrand Factor / chemistry
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor
  • Ristocetin
  • Collagen