About Von Willebrand Disease — What is VWD
Types and Symptoms of von Willebrand Disease
Diagnosing von Willebrand Disease
Treatment Options
Humate-P
Humat-P for VWD — High-Quality VWF
Using Humate-P
The CSL Behring Commitment
Resources & Tools
News & Events
Important Safety Information
Prescribing Information
Dosing Calculator
VWF Densitometric Analysis
Request More Information
For Patients

Diagnosing von Willebrand Disease (VWD)

Accurately diagnosing VWD can be a challenge1

  • Because most cases of VWD are mild, many people are asymptomatic or fail to report symptoms—thus, the majority are currently undiagnosed2
  • It is essential to establish a family medical history—however, family history alone is not always adequate to identify VWD3
  • In order to prescribe the best treatment option, physicians who suspect VWD must determine the type and subtype through specific blood tests

The need for specific diagnostic assays
Standard assays for diagnosing a coagulation disorder—eg, prothrombin time and activated partial thromboplastin time—are generally inadequate for detecting mild forms of VWD.3,4

Please see Important Safety Information about Humate-P®

Specific assays useful for accurately diagnosing VWD:


 Diagnostic assay Purpose of assay
VWF activity (VWF:RCo) Provides a quantitative measurement of VWF function5
VWF multimers Analyzes the quality and provides semiquantitative measurement of VWF multimers—essential for determining subtype6,7
FVIII clotting activity (FVIII:C) Determines the degree of FVIII activity5
VWF antigen (VWF:Ag) Measures the quantity of VWF2,6
Ristocetin-induced platelet aggregation (RIPA) Provides a semiquantitative measurement of VWF function8


Important Safety Information

Antihemophilic Factor/von Willebrand Factor Complex (Human), Dried, Pasteurized, Humate-P® is indicated in adult patients for treatment and prevention of bleeding in 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 contraindicated in individuals with a history of anaphylactic or severe systemic response to antihemophilic factor or von Willebrand factor preparations or to any of its components.

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

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

Although few adverse reactions have been reported the most common are allergic-anaphylactic reactions, including urticaria, chest tightness, rash, pruritus, edema, shock, chills and fever, and hypervolemia. For patients undergoing surgery, the most common adverse reactions are postoperative wound or injection-site bleeding.

Please see accompanying full prescribing information for Humate-P®.

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. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683-694.
  2. Rick ME. Diagnosis and management of von Willebrand's syndrome. Med Clin North Am. 1994;78:609-623.
  3. Katsanis E, Luke K-H, Hsu E, Li M, Lillicrap D. Prevalence and significance of mild bleeding disorders in children with recurrent epistaxis. J Pediatr. 1988;113:73-76.
  4. Werner EJ, Abshire TC, Giroux DS, Tucker EL, Broxson EH. Relative value of diagnostic studies for von Willebrand disease. J Pediatr. 1992;121:34-38.
  5. White GC II, Montgomery RR. Clinical aspects of and therapy for von Willebrand disease. In: Hoffman R, Benz EJ Jr, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, eds. Hematology: Basic Principles and Practice. 3rd ed. New York: Churchill Livingstone Inc; 2000:1946-1958.
  6. Holmberg L, Nilsson IM. von Willebrand’s disease. Eur J Haematol. 1992;48:127-141
  7. Santoro SA, Eby CS. Laboratory evaluation of hemostatic disorders. In: Hoffman R, Benz EJ Jr, Shattil SJ, Furie B, Cohen HJ, Silberstein LE, eds. Hematology: Basic Principles and Practice. 2nd ed. New York: Churchill Livingstone Inc; 1995:1622-1632.
  8. Vosburgh E. Rational intervention in von Willebrand’s disease. Hosp Pract. March 30, 1993:31-48.