Laboratory diagnosis of von Willebrand disease

Int J Lab Hematol. 2015 May;37 Suppl 1(Suppl 1):11-7. doi: 10.1111/ijlh.12345.

Abstract

Von Willebrand disease (VWD) is considered the most common inherited bleeding disorder and may also be the most difficult to diagnose. Clinical symptoms of VWD include predominantly mild mucosal bleeding; surgical bleeding may occur with specific challenges and joint bleeding can occur in the most severe forms. A family history either of diagnosed VWD or of bleeding symptoms is typically present. Laboratory diagnosis requires a series of assays of von Willebrand factor (VWF) quantity and function, and factor VIII activity, with no single straightforward diagnostic test available to either confirm or exclude the diagnosis. Newer assays of VWF function are becoming more available and useful in determining the laboratory diagnosis of VWD.

Keywords: Von Willebrand disease; platelets; von Willebrand factor.

Publication types

  • Review

MeSH terms

  • Clinical Laboratory Techniques / methods*
  • Factor VIII / metabolism*
  • Humans
  • Reproducibility of Results
  • Ristocetin / metabolism
  • Sensitivity and Specificity
  • von Willebrand Diseases / blood
  • von Willebrand Diseases / classification
  • von Willebrand Diseases / diagnosis*
  • von Willebrand Factor / metabolism*

Substances

  • von Willebrand Factor
  • Ristocetin
  • Factor VIII