Mechanism of Action

Leverage the hemostatic potential of HMW multimers

The size of VWF multimers directly correlates with their in vitro function. 1,2

  • High molecular weight (HMW) VWF multimers are most essential for primary hemostasis, whereas low molecular weight (LMW) multimers are less functionally active2
  • HMW-VWF multimers have been shown to be associated with increased hemostatic activity3
  • HMW-VWF multimers are associated with shortened bleeding time3

The process of blood coagulation has two distinct stages of hemostasis: primary hemostasis and secondary hemostasis. While factor VIII (FVIII) has a role in secondary hemostasis, von Willebrand factor protein is a key component in primary hemostasis.

People with von Willebrand disease (VWD) have an insufficient amount of VWF protein in the blood, or VWF that is defective. Because VWF is essential in primary hemostasis, an insufficient amount or a supply of defective VWF interferes with proper platelet adherence or aggregation. The mainstay of VWD treatment is the replacement of the deficient VWF protein. This results in shortened bleeding time and correction of the coagulation abnormality.

The VWF profile of Humate-P strongly correlates to hemostatic activities during the stages of hemostasis2,4

HMW-VWF multimers promote:

  • Effective collagen binding
  • Effective platelet adhesion
  • Effective platelet aggregation

Successful completion of both stages of hemostasis results in the final step of the clotting process, the formation of a stable hemostatic plug.

As close as multimer patterns get to normal human plasma with appropriate FVIII levels

Like normal human plasma, Humate-P contains a high percentage of HMW-VWF multimers, and can correct the hemostatic defect in patients with VWD.1,3

View an animated densitometric analysis comparing various VWF/FVIII concentrates and normal human plasma.

Important Safety Information

Antihemophilic Factor/von Willebrand Factor Complex (Human), 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 risk factors for thrombosis.

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

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

Please see full prescribing information.

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The information provided here is primarily intended for use by physicians and other 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.

Treatment for VWD patients

© CSL Behring 2018. The product information presented on this site is intended for US residents only. 11-HUM-082

References

  1. Berntorp E, Nilsson IM. Biochemical and in vivo properties of commercial virus-inactivated factor VIII concentrates. Eur J Haematol. 1988;40:205-214.
  2. Budde U, Metzner HJ, Müller H-G. Comparative analysis and classification of von Willebrand factor/factor VIII concentrates: impact on treatment of patients with von Willebrand disease. Semin Thromb Hemost. 2006;32:626–635.
  3. Metzner HJ, Hermentin P, Cuesta-Linker T, Langner S, Müller H-G, Friedbold J. Characterization of factor VIII/von Willebrand factor concentrates using a modified method of von Willebrand factor multimer analysis. Haemophilia.1998;4(suppl 3):25-32.
  4. Lethagen S, Carlson M, Hillarp A. A comparative in vitro evaluation of six von Willebrand factor concentrates. Haemophilia. 2004;10:243-249.