von Willebrand Disease: Diagnostic Strategies and Treatment Options

Christopher J Ng; Jorge Di Paola

doi:10.1016/j.pcl.2018.02.004

von Willebrand Disease: Diagnostic Strategies and Treatment Options

Pediatr Clin North Am. 2018 Jun;65(3):527-541. doi: 10.1016/j.pcl.2018.02.004.

Authors

Christopher J Ng¹, Jorge Di Paola²

Affiliations

¹ Department of Pediatrics, University of Colorado, Children's Hospital Colorado, 12800 East 19th Avenue, Research Center 1 North, MS 8302, Aurora, CO 80111, USA.
² Department of Pediatrics, University of Colorado, Children's Hospital Colorado, 12800 East 19th Avenue, Research Center 1 North, MS 8302, Aurora, CO 80111, USA. Electronic address: jorge.dipaola@ucdenver.edu.

PMID: 29803281
DOI: 10.1016/j.pcl.2018.02.004

Abstract

von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. Since its first description in 1926, the diagnosis and management of VWD has significantly improved due to increasing scientific knowledge of the genetics and biology of von Willebrand factor (VWF). This article reviews the molecular structure and function of VWF as well as the clinical symptoms, laboratory-based diagnostic workup, and classification schema for VWD. It highlights current treatment options and state-of-the art research in VWF and VWD.

Keywords: Mucocutaneous bleeding; von Willebrand disease; von Willebrand factor.

Publication types

Review

MeSH terms

Blood Coagulation Factors / therapeutic use
Deamino Arginine Vasopressin / therapeutic use
Hemostatics / therapeutic use
Humans
von Willebrand Diseases / diagnosis*
von Willebrand Diseases / etiology
von Willebrand Diseases / therapy*

Substances

Blood Coagulation Factors
Hemostatics
Deamino Arginine Vasopressin