What is pseudo von Willebrand disease?
Pseudo (platelet-type)-von Willebrand disease is an autosomal dominant bleeding disorder caused by the hyperfunction of a receptor on the platelet surface. The abnormal receptor, glycoprotein Ib, displays increased affinity for its ligand, von Willebrand factor.
What is the difference between hemophilia and von Willebrand disease?
Unlike hemophilia, people with VWD rarely bleed into their joints. Less than 8% of patients report joint bleeding. VWD is more likely to cause easy bruising and nosebleeds. Women with VWD can have very heavy menstrual periods.
What is the most common breed to have von Willebrand’s disease?
At least thirty different breeds are affected, but the Doberman Pinscher is the breed with the highest incidence of vWD. Of 15,000 Dobermans screened in a research study, more than 70% were found to be carriers of the disease.
What is von Willebrand type 2B?
Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder caused by changes in von Willebrand factor (VWF) that enhance binding of VWF to GPIb on platelets.
What causes VWF?
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 factor is VWF?
von Willebrand factor (VWF) is a large adhesive glycoprotein required for platelet adhesion to subendothelium at the site of vessel injury, platelet aggregation to form the platelet plug, and stabilization of factor VIII (FVIII) in the circulation. Deficiency or defect of VWF leads to von Willebrand disease (VWD).
What is Factor VII deficiency?
Factor VII deficiency is a rare bleeding disorder that varies in severity among affected individuals. The signs and symptoms of this condition can begin at any age, although the most severe cases are apparent in infancy. However, up to one-third of people with factor VII deficiency never have any bleeding problems.
Is von Willebrand’s hemophilia?
Von Willebrand disease is the most common type of bleeding disorder in the U.S. It is similar to another blood disorder called hemophilia, though it is usually less severe than hemophilia. Von Willebrand disease affects 1% of the U.S. population.
What breeds are affected by von Willebrand disease?
Von Willebrand’s Disease in Dogs
- Doberman Pinscher.
- Shetland Sheepdog.
- Miniature Schnauzer.
- Golden Retriever.
- Rottweiler.
- German Shepherd.
- Standard Poodle.
- German Shorthaired Pointer.
What is mucocutaneous bleeding?
Mucocutaneous hemorrhage are typically characterized by spontaneous or slightly traumatic bleeding resulting from the coagulopathies and hemostatic disorders, which means that circulating blood infiltrating into the skin or submucosal tissue from the capillaries.
When to use biostate for von Willebrand disease?
Both FVIII and VWF are blood proteins that are essential for normal blood clotting. Biostate® is used in patients with von Willebrand Disease (VWD), a bleeding disorder resulting from low levels of VWF or abnormal VWF.
How does von Willebrand disease ( vWD ) affect the body?
Deficiency of vWF causes inadequate platelet adhesion and secondary deficiency of Factor FVIII. Affects males and females equally. Characterised by easy bruising, bleeding from mucous membranes (particularly epistaxis, oral mucosa, menorrhagia) and post-op bleeding. There are three main phenotypes of vWD.
How is Factor replacement therapy used to treat vWF?
Factor Replacement Therapy. Recombinant VWF (such as Vonvendi®) and medicines rich in VWF and factor VIII (for example, Humate P®, Wilate®, Alphanate®, or Koate DVI®) are used to treat people with more severe forms of VWD or people with milder forms of VWD who do not respond well to the nasal spray.
When to use von Willebrand factor / FVIII plasma concentrate?
Von Willebrand Factor/FVIII Plasma Concentrate (Biostate®) A human plasma-derived product, available from blood bank. May be required in Type 1 vWD if severe bleeding or unresponsive to DDAVP. Used to treat bleeding in patients with Type 2 and Type 3 vWD.