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Types and Symptoms of von Willebrand Disease (VWD)

Types of VWD
There are 3 main types of VWD. Types 1 and 3 refer to quantitative defects in VWF; type 2 is a qualitative defect.1

  • Type 1—most common and mildest form (as many as 80% of cases), type 1 is marked by a mild to moderate reduction in VWF1,2
  • Type 2—divided into four subtypes: 2A, 2B, 2M, and 2N3
    — 2A: The absence of high molecular weight (HMW) VWF results in a loss of platelet-dependent function1
    — 2B: Defective VWF causes overactive binding of A1 platelet receptor, resulting in a lack of high molecular weight multimers (HMWM)1
    — 2M: Similar to type 2B, but platelets do not clump.1 2M is not associated with multimer defects3
    — 2N: Defective VWF cannot bind to FVIII.3 Laboratory tests show similar results for type 2N and mild hemophilia A, which can lead to misdiagnoses4
  • Type 3—The rarest and most severe form of VWD, type 3 has almost no detectable VWF levels, resulting in lower factor VIII levels3

Symptoms of VWD5

  • Easy bruising
  • Epistaxis
  • Menorrhagia
  • Prolonged bleeding following surgery, dental work, childbirth, or injury
  • In severe cases, gastrointestinal bleeding or hemarthrosis


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).

Please see full prescribing information.

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.

The information provided herein is solely for use by physicians and healthcare professionals in the United States. The CSL Behring product listed may not have been approved in other countries and may not be available everywhere.

References

  1. Lillicrap D. The basic science, diagnosis and clinical management of von Willebrand disease. Treatment of Hemophilia. Montreal, Canada: World Federation of Hemophilia; 2004;35:1-11.
  2. The “Other” Bleeding Disorder. Available at: http://www.haemophilia.org.za/Vonwil2.htm. Accessed April 15, 2005.
  3. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683-694.
  4. Gill JC. Pathophysiology of von Willebrand disease and the new classification system. In: W. Keith Hoots, moderator. Diagnosis and Management of Severe von Willebrand Disease. Philadelphia: The National Hemophilia Foundation Satellite Symposium; October 13, 1995.
  5. National Heart, Lung, and Blood Institute Diseases and Conditions Index. What are the signs and symptoms of von Willebrand disease? Available at: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_SignsAndSymptoms.html. Accessed September 17, 2005.
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