Get the Facts on von Willebrand Disease.

von Willebrand factor:
The main factor behind von Willebrand disease
The underlying cause of von Willebrand disease (VWD) is a deficiency or defect in von Willebrand factor (VWF), a multimeric protein important for clotting. Explore the pathophysiology and classification of VWD.1,2

von Willebrand disease mechanism: VWF is critical for hemostasis and thrombus formation and plays 2 key roles2:

Diagram showing binding to collagen and platelets to facilitate platelet plug formation.

Binding to collagen and platelets to facilitate platelet plug formation2,3

Diagram showing protecting factor VIII from clearance.

Binding to factor VIII (FVIII) to protect it from clearance and stabilize plasma FVIII levels2,3

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Because VWF protects FVIII in plasma, VWF deficiency or defect leads to increased FVIII clearance, resulting in low FVIII levels in some patients with VWD. Low levels of FVIII (<30-40 IU/dL) may lead to prolonged activated partial thromboplastin time (aPTT)2,4,5,6

There are 3 types of VWD7
Type 1

VWF is deficient

Type 2

VWF is defective

Type 3

VWF is almost absent

Depending on type, VWD can be inherited in either an autosomal recessive or dominant manner.8
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An important fact to note about all 3 types of VWD is that they only impact production of functional VWF—VWD itself does not impact patients’ ability to produce FVIII2,9-11

Bleeds happen in all types of VWD2

Because VWD patients lack VWF multimer protein, an important protein for clotting, they are prone to bleeds. Patients with VWD Types 1, 2, and 3 have all reported a range of bleeding episodes.

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Severe Type 3 patients experience frequent bleeds

In an on-demand clinical trial where VONVENDI was being investigated, 21 adult patients with severe Type 3 VWD (VWF: Ag≤3%) experienced a median (range) of 5.7 (0.0, 36.5) bleeds in a year that required treatment.12

Could VONVENDI be right for your appropriate adult patients?

Learn more
VWF factor replacement can be used in 3 ways for adults with VWD13-15
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To treat acute bleeding episodes

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To manage bleeding around surgery

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Prophylaxis (severe type 3)

To proactively reduce the number of bleeds

2021 ASH/ISTH/NHF/WFH treatment guidelines have provided insight into when prophylaxis may be considered for recommendation in appropriate VWD patients.16