While the more common types of von Willebrand’s disease are inherited, it is also possible to develop the condition from an autoimmune disease such as lupus or cancer, or as a result of taking some medications. This is called acquired von Willebrand’s disease.
What does VWF do to platelets?
Von Willebrand factor (VWF) is a large multimeric glycoprotein that performs two critical functions in primary hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion , and under high shear conditions, it promotes platelet aggregation.
Can von Willebrand disease cause low platelet count?
Affected individuals may also experience a low platelet count (thrombocytopenia). Individuals with PT-VWD are difficult to distinguish from individuals VWD type 2B. PT-VWD is caused by mutations of the GP1BA gene and is inherited as an autosomal dominant disorder.
How do platelets and von Willebrand factor interact?
Von Willebrand factor (VWF) is a large multimeric glycoprotein that performs two critical functions in primary hemostasis: it acts as a bridging molecule at sites of vascular injury for normal platelet adhesion , and under high shear conditions, it promotes platelet aggregation.
Does von Willebrand factor cause platelet aggregation?
The von Willebrand factor (vWF) has two major well-established biological functions: mediating adhesion and aggregation of platelets , and acting as a consort for the essential blood clotting factor: factor VIII [1].
Does von Willebrand go away?
Von Willebrand factor helps blood cells stick together (clot) when you bleed. If there’s not enough of it or it does not work properly, it takes longer for bleeding to stop. There’s currently no cure for VWD , but it does not usually cause serious problems and most people with it can live normal, active lives.
Can I donate blood if I have von Willebrand’s disease?
But I have von Willebrand Disease (VWD), can’t I donate blood? Some blood collection centers will accept donations from people with VWD who have never had a major bleed or required treatment for their VWD. Call first and talk to a donation counselor.
What causes the release of von Willebrand factor?
The usual cause of von Willebrand disease is an inherited abnormal gene that controls von Willebrand factor — a protein that plays a key role in blood clotting.
What activates von Willebrand?
The protein is activated by flowing blood and thus has the potential to stimulate the formation of blood clots even in the absence of vessel damage. In order to prevent unnecessary clotting, VWF is regulated by an enzyme known as ADAMTS13.
What is Bernard Soulier syndrome?
General Discussion. Bernard-Soulier syndrome (BSS) is a rare inherited disorder
Do platelets release VWF?
After platelet contact, platelet vWF is released , binds to the glycoprotein IIb/IIIa complex, and forms a bridge between the subendothelial surface and the platelet, which initiates and supports platelet spreading.
Is von Willebrand a platelet disorder?
Platelet-type von Willebrand disease (PT-VWD), also known as pseudo-von Willebrand disease, is an extremely rare genetic disorder characterized by prolonged bleeding time . Unlike VWD, the underlying defect affects platelets, which have an unusually high binding affinity to VWF.
What is the normal range for von Willebrand factor?
In the general population, the mean level of plasma VWF is 100 IU per dL, with a normal reference range between 50 and 200 IU per dL .
At what age is von Willebrand disease diagnosed?
76% of men with VWD had been diagnosed by age 10 , but 50% of women with VWD were not diagnosed until after age 12.
Can von Willebrand disease get worse?
This protein is called the von Willebrand factor. It helps your blood to clot by helping the platelets stick together. The disease is mild in most people. It can stay the same or get better or worse as you get older .
What happens if you have von Willebrand disease?
People with von Willebrand disease sometimes experience heavier-than-normal bleeding from injury, surgery , and, in women, menstrual flow and childbirth. This bleeding can cause health problems including pain and anemia (low number of red blood cells). In rare cases, the bleeding can lead to death.
