Von Willebrand Disease: Current Status of Diagnosis and Management

Hematol Oncol Clin North Am. 2021 Dec;35(6):1085-1101. doi: 10.1016/j.hoc.2021.07.004. Epub 2021 Aug 13.

Abstract

Von Willebrand disease (VWD) is a common bleeding disorder, affecting male and female individuals equally, that often manifests in mucosal bleeding. VWD can be secondary to a quantitative (Type 1 and Type 3) or qualitative (Type 2) defects in Von Willebrand factor (VWF). Initial testing includes VWF antigen, as well as a platelet binding assay to differentiate between qualitative and quantitative defects. Further subtyping requires additional testing and is needed to ensure appropriate treatment. Desmopressin, antifibrinolytics, hormonal treatments for heavy menstrual bleeding, and VWF concentrates are commonly used in the treatment of VWD.

Keywords: Bleeding; Coagulation factors; von Willebrand disease; von Willebrand factor.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Blood Platelets
  • Female
  • Humans
  • Male
  • von Willebrand Diseases* / diagnosis
  • von Willebrand Diseases* / drug therapy
  • von Willebrand Factor

Substances

  • von Willebrand Factor