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Von Willebrand disease in the United States: perspective from the Zimmerman program. Ann Blood 2018 Jan;3

Date

08/24/2018

Pubmed ID

30135959

Pubmed Central ID

PMC6100783

DOI

10.21037/aob.2017.12.05

Abstract

This article will discuss the diagnosis and management of von Willebrand disease (VWD) in the United States and results from the Zimmerman Program, a national study of VWD. An algorithm is presented to show how we currently approach diagnostic testing for VWD, including the potential replacement of the ristocetin cofactor assay with a new von Willebrand factor (VWF)-GPIb binding assay. Results from the Zimmerman Program type 1 cohort are presented, including the findings that genetic defects in the VWF gene are most common with VWF levels <30 IU/dL, but bleeding symptoms were present across the entire cohort regardless of VWF level. Typical management of VWD patients is also discussed, including the use of desmopressin and VWF concentrates. Despite these advances, there remain several areas of VWD where more research is required to optimize treatment.

Author List

Flood VH, Abshire TC, Christopherson PA, Friedman KD, Cox Gill J, Montgomery RR, Haberichter SL, Zimmerman Program Investigators

Authors

Veronica H. Flood MD Professor in the Pediatrics department at Medical College of Wisconsin
Kenneth D. Friedman MD Professor in the Medicine department at Medical College of Wisconsin
Robert R. Montgomery MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin