Types of von Willebrand Disease (VWD)

The type of VWD depends on whether a person’s von Willebrand factor (VWF) doesn’t work properly, or if there is not enough of it.

There are three main types of VWD:

Type of VWD	Purpose of assay	% of VWD Patients
Type 1	Not enough VWF	About 80%^1,2
Type 2 (includes subtypes 2A, 2B, 2N, and 2M)	VWF doesn’t work properly	About 20%
Type 3	Very little or no VWF	Very rare

Type 1 means that the VWF works properly, but there is not enough of it. This is the most common type of VWD, making up 80% of all cases.^1,2

Type 2 is when the VWF does not work properly, which means the blood will not clot properly. About 20% of people with VWD have Type 2. There are four different kinds (or subtypes) of Type 2, each referring to a specific kind of defect in the von Willebrand factor. The subtypes are Type 2A, 2B, 2M, and 2N.

Type 3 is an almost complete lack of VWF. Type 3 is the rarest, but most severe, form of VWD (it affects 1–3 people per million).³

Symptoms of VWD

Key symptoms of VWD include bleeding a lot during dental work or bruising very easily. Yet many people with undiagnosed VWD will dismiss these symptoms or say, “This is normal for my family. We all bruise very easily.” Another key symptom is very heavy menstrual periods (menorrhagia) in women. Yet, many women who have von Willebrand disease mistakenly believe that this heavy bleeding is normal and untreatable.

Important Safety Information

Humate-P is indicated for treatment and prevention of bleeding in adult patients with hemophilia A (classical hemophilia). Humate-P is also indicated in adult and pediatric patients with von Willebrand disease (VWD) for (1) treatment of spontaneous and trauma-induced bleeding episodes, and (2) prevention of excessive bleeding during and after surgery. This applies to patients with severe VWD, and patients with mild and moderate VWD for whom use of desmopressin is known or suspected to be inadequate. Humate-P is not indicated for the prophylaxis of spontaneous bleeding episodes.

Humate-P is contraindicated in individuals with a history of anaphylactic or severe systemic response to antihemophilic factor or von Willebrand factor preparations.

Monitor for intravascular hemolysis and decreasing hematocrit values in patients with A, B, and AB blood groups who are receiving large or frequent doses. Also monitor VWF:RCo and FVIII levels in VWD patients, especially those undergoing surgery.

Thromboembolic events have been reported in VWD patients receiving coagulation factor replacement. Caution should be exercised and antithrombotic measures considered, particularly in patients with known risk factors for thrombosis.

Humate-P is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

In patients receiving Humate-P in clinical studies for treatment of VWD, the most commonly reported adverse reactions observed by >5% of subjects are allergic-anaphylactic reactions, including urticaria (hives), chest tightness, rash, pruritus (itching), and edema (swelling). For patients undergoing surgery, the most common adverse reactions are postoperative wound or injection-site bleeding, and epistaxis (nosebleed).

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.