von Willebrand Disease in Pediatrics: Evaluation and Management

Hematol Oncol Clin North Am. 2019 Jun;33(3):425-438. doi: 10.1016/j.hoc.2019.01.010.

Abstract

Advances in the use of standardized bleeding assessment tools, functional laboratory assays, and genetic testing have all improved the ability of hematologists to more accurately diagnose von Willebrand disease (VWD) in patients presenting with easy bruising or bleeding. This article reviews these recent advances, as well as currently available treatment options, including desmopressin, antifibrinolytics, and both plasma-based and recombinant von Willebrand factor products. The diagnosis of VWD remains complex and this article provides an understanding of the various subtypes and phenotypes of VWD, as well as the appropriateness and interpretation of the many available laboratory tests.

Keywords: Diagnosis; Management; Pediatric; von Willebrand disease.

Publication types

  • Review

MeSH terms

  • Algorithms
  • Blood Coagulation Tests / methods
  • Child
  • Genetic Testing / methods*
  • Hemorrhage / diagnosis*
  • Hemorrhage / genetics
  • Humans
  • Pediatrics / methods*
  • Phenotype
  • von Willebrand Diseases / diagnosis
  • von Willebrand Diseases / genetics
  • von Willebrand Diseases / therapy*
  • von Willebrand Factor / genetics
  • von Willebrand Factor / metabolism*

Substances

  • von Willebrand Factor