You’re going to want to know the type and severity of the disease (see notes below). It is also important to gauge your approach based on the surgery being performed the likelihood of high blood loss.

For Type I (majority of the cases), Platelet and Von Willebrand Factor (vWF) stores will be normal – the problem is in the release of vWF.

Treatment: The most reliable treatment is blood products (cryoprecipitate or purified concentrates containing vWF). All types of vWF benefit from cryoprecipitate and fresh frozen plasma (FFP). Other treatments depend on the type of vWF. Below are some treatments that are more type-specific:

Type 1: Desmopressin (DDAVP) 0.3-0.5 mcg/kg IV (watch for hypotension). DDAVP optimizes plasma levels of endogenous vWF.
Type 3: Purified Factor VIII/vWF concentrate.

The effects of these treatments only last 12-24 hours.

This is the most common inherited disorder affecting platelet function.

VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process.

Signs/Symptoms are primarily BLEEDING: mucocutaneous bleeding (epistaxis), easy bruising, menorrhagia, gingival and GI bleeding, prolonged PTT, hemarthrosis, and deep tissue bleeds.

Type I is a quantitative defect in plasma vWF release. It is by far the most common (80% of cases).
Type II is a qualitative problem with vWF.
Type III is a virtual absence of circulating vWF antigen. There are low levels of both vWF and Factor VIII