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Thromb Haemost 2020; 120(08): 1159-1165
DOI: 10.1055/s-0040-1713636
Review Article

The Effect of Age on von Willebrand Factor and Bleeding Symptoms in von Willebrand Disease

Craig D. Seaman
1   Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
2   Hemophilia Center of Western Pennsylvania, Pittsburgh, Pennsylvania, United States
,
Margaret V. Ragni 
1   Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States
2   Hemophilia Center of Western Pennsylvania, Pittsburgh, Pennsylvania, United States
› Author Affiliations Funding This study was funded by the University of Pittsburgh Cancer Institute.
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Abstract

von Willebrand disease (VWD) is a quantitative or qualitative defect in von Willebrand factor (VWF) resulting in mucocutaneous bleeding symptoms and hemorrhage following hemostatic challenges, such as trauma or surgery. VWD-specific therapy, DDAVP (1-desamino-8-D-arginine vasopressin) and VWF concentrates, is necessary periprocedurally to ensure adequate hemostasis. The aging VWD patient may complicate this matter. The plasma concentration of many coagulation proteins, including VWF, increases with age. While it has been established that VWF levels increase with age in a healthy population, emerging research demonstrates this occurs in certain subtypes of VWD, too. Thus, the management of periprocedural VWD-specific therapy in the aging VWD patient is problematic when VWF levels increase over time to normal, and hematologists are left with uncertainty regarding whether or not periprocedural VWD-specific therapy is still necessary. In this article, we will review the current state of the literature regarding the effect of age on VWF levels in the healthy population and VWD while exploring possible etiologies for this phenomenon. Further, we will detail how this affects bleeding symptoms and highlight what research remains to be done to optimize care in this patient population.

Keywords

von Willebrand disease - von Willebrand factor - age - bleeding score

Authors' Contributions

C.D.S. and M.V.R. researched the topic, analyzed the data, formulated the conclusions, and wrote the paper.




Publication History

Received: 17 April 2020

Accepted: 15 May 2020

Article published online:
06 July 2020

Georg Thieme Verlag KG
Stuttgart · New York

 

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