Occurrence rates of von Willebrand disease among people receiving care in specialized treatment centres in the United States

Haemophilia ◽  
2021 ◽  
Author(s):  
John Michael Soucie ◽  
Connie H. Miller ◽  
Vanessa R. Byams ◽  
Amanda B. Payne ◽  
Karon Abe ◽  
...  
Keyword(s):  
United States ◽  
The United States ◽  
Von Willebrand Disease ◽  
Specialized Treatment ◽  
Von Willebrand

scholarly journals Von Willebrand disease in the United States: perspective from the Zimmerman program

2018 ◽  
Vol 3 ◽  
pp. 7-7 ◽  
Author(s):  
Veronica H. Flood ◽  
◽  
Thomas C. Abshire ◽  
Pamela A. Christopherson ◽  
Kenneth D. Friedman ◽  
...  
Keyword(s):  
United States ◽  
The United States ◽  
Von Willebrand Disease ◽  
Von Willebrand

scholarly journals von Willebrand Disease in the United States: A Perspective from Wisconsin

2011 ◽  
Vol 37 (05) ◽  
pp. 528-534 ◽  
Author(s):  
Veronica Flood ◽  
Joan Gill ◽  
Kenneth Friedman ◽  
Daniel Bellissimo ◽  
Sandra Haberichter ◽  
...  
Keyword(s):  
United States ◽  
The United States ◽  
Von Willebrand Disease ◽  
Von Willebrand

scholarly journals What have we learned from large population studies of von Willebrand disease?

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 670-677 ◽  
Author(s):  
Robert R. Montgomery ◽  
Veronica H. Flood
Keyword(s):  
United States ◽  
The United States ◽  
Von Willebrand Disease ◽  
Assessment Tools ◽  
Platelet Glycoprotein ◽  
Hemorrhagic Disease ◽  
Collagen Binding ◽  
Functional Variants ◽  
Plasma Factor ◽  
Von Willebrand

Abstract Von Willebrand factor (VWF) is a critical regulator of hemostatic processes, including collagen binding, platelet adhesion, and platelet aggregation. It also serves as a carrier protein to normalize plasma factor VIII synthesis, release, and survival. While VWF protein measurements by immunoassay are reasonably comparable between institutions, the measurement of VWF ristocetin cofactor activity (VWF:RCo) has significant variability. Other tests of VWF function, including collagen binding or platelet glycoprotein IIb-IIIa binding, are not universally available, yet these functional defects may cause major bleeding even with normal VWF antigen (VWF:Ag) and VWF:RCo assays. This results in both the overdiagnosis and underdiagnosis of VWD. Newer assays of VWF function (using recombinant glycoprotein Ib rather than whole platelets) have been developed that may improve interlaboratory variability. Some of these tests are not uniformly available and may not be licensed in the United States. Large longitudinal studies of VWF in von Willebrand disease (VWD) patients are not available. Patients are sometimes diagnosed with a single diagnostic VWF panel. Plasma VWF levels increase with age, but it is not clear if this results in less bleeding or whether different normal ranges should be used to identify age-related decreases in VWF. In order to quantitatively compare bleeding symptoms in VWD patients and normal individuals, recent studies in the European Union, Canada, United Kingdom, Holland, and the United States have used semiquantitative bleeding assessment tools (BATs). Even with careful centralized testing, including functional assays of VWF, addition of a BAT does not solve all of the problems with VWD diagnosis. No matter where the line is drawn for diagnosis of VWD, VWF is still a continuous variable. Thus, VWD can be a severe hemorrhagic disease requiring frequent treatment or a mild condition that may not be clinically relevant. As will be discussed by Dr. Goodeve in her presentation, genetics has helped us to diagnose type 2 functional variants of VWD but has not been helpful for the many patients who are at the interface of normal and low VWF and carry the possible diagnosis of type 1 VWD. The hematologist’s management of patients with reduced levels of VWF still requires both the art and science of clinical medicine.

scholarly journals Complications of Hysterectomy in Women with Von Willebrand Disease.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3399-3399
Author(s):  
Andra H James ◽  
Evan R Myers ◽  
Chad Cook ◽  
Ricardo Pietrobon
Keyword(s):  
Case Reports ◽  
Postoperative Bleeding ◽  
Case Series ◽  
The United States ◽  
Von Willebrand Disease ◽  
Heavy Menstrual Bleeding ◽  
Menstrual Bleeding ◽  
Bleeding Complications ◽  
Risk Of Bleeding ◽  
Von Willebrand

Abstract Background: Case reports and small case series suggest that women with von Willebrand disease (VWD) are at a very high risk of bleeding complications with hysterectomy. Because the procedure may be beneficial to women who suffer from heavy menstrual bleeding, an understanding of the true risks involved is essential for appropriate decision making. Objectives: To estimate the incidence of bleeding and other complications in women with VWD who undergo hysterectomy. Methods: The United States Nationwide Inpatient Sample (NIS) from the Healthcare Cost and Utilization Project of the Agency for Healthcare Research and Quality for the years 1988–2004 was queried for all hysterectomies for nonmalignant conditions. Data were analyzed based on the NIS sampling design. Bivariate analyses were used to examine the differences between women with and without VWD. Multivariate analysis was used to adjust for potential confounders among women who underwent hysterectomy for heavy menstrual bleeding. Results: 545 of the 1,358,133 hysterectomies were to women with VWD. Women with VWD were significantly more likely to experience intraoperative and postoperative bleeding (2.75% versus 0.89%, p < 0.001) and require transfusion (7.34% versus 2.13%, p < 0.001) than women without VWD. One woman with VWD died (odds ratio = 28.49). Conclusions: While the risk of bleeding complications from hysterectomy in women with VWD is smaller than previously reported, women with VWD did experience significantly more bleeding complications than women without VWD. Nonetheless, for women who have completed childbearing, the risks of hysterectomy may be acceptable.

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