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Advances in the diagnosis and treatment of Von Willebrand disease. Blood 2017 11 30;130(22):2386-2391

Date

12/01/2017

Pubmed ID

29187375

Pubmed Central ID

PMC5709787

DOI

10.1182/blood-2017-05-782029

Scopus ID

2-s2.0-85036542444   35 Citations

Abstract

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 VH

Author

Veronica H. Flood MD Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Animals
Collagen
Genetic Variation
Hemorrhage
Humans
Platelet Glycoprotein GPIb-IX Complex
von Willebrand Diseases
von Willebrand Factor