Advances in the diagnosis and treatment of Von Willebrand disease. Blood 2017 Nov 30;130(22):2386-2391
Date
12/01/2017Pubmed ID
29187375Pubmed Central ID
PMC5709787DOI
10.1182/blood-2017-05-782029Scopus ID
2-s2.0-85036542444 (requires institutional sign-in at Scopus site) 72 CitationsAbstract
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, yet diagnosis and management remain challenging. Development and use of bleeding assessment tools allows for improved stratification of which patients may require further assessment and which patients are most likely to require treatment of their VWD. New options for laboratory assessment of von Willebrand factor (VWF) activity include a new platelet-binding assay, the VWF:GPIbM, which is subject to less variability than the ristocetin cofactor activity assay, and collagen-binding assays that provide insight into a different function of VWF. Genetic testing may be helpful in some cases where a type 2 VWD variant is suspected but is usually not helpful in type 1 VWD. Finally, treatment options for VWD are reviewed, including the use of recombinant VWF. Despite these advances, still more work is required to improve diagnosis, treatment, and quality of life for affected patients.
Author List
Sharma R, Flood VHAuthor
Veronica H. Flood MD Chief, Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AnimalsCollagen
Genetic Variation
Hemorrhage
Humans
Platelet Glycoprotein GPIb-IX Complex
von Willebrand Diseases
von Willebrand Factor
