octapharma
For the treatment of VWD

Types of von Willebrand Disease (VWD)

von Willebrand disease (VWD) is divided into three main types, classified according to von Willebrand factor (VWF) defect.1 Type 1 involves a partial quantitative deficiency of VWF. Type 2 involves the qualitative VWF defects, including structural or functional defects of the VWF protein. Type 3 involves a complete deficiency of VWF. In general, the severity of bleeding worsens from type 1 to type 3 disease.1

Classification of VWD2

Type Description
% of patients
with VWD3
1 Partial quantitative deficiency of VWF ~ 60% - 80%
2 Qualitative VWF defects ~ 10% - 30%
  2A
Decreased VWF-dependent platelet adhesion and a selective
deficiency of high-molecular-weight multimers
 
2B Increased affinity for platelet glycoprotein Ib
2M
Decreased VWF-dependent platelet adhesion without selective
deficiency of high-molecular-weight multimers
2N Markedly decreased binding affinity for Factor VIII
3 Virtual complete deficiency of VWF ~ 1% - 5%

 

Initial hemostasis laboratory evaluation usually includes a platelet count and complete blood count (CBC), partial thromboplastin time (PTT), prothrombin time (PT), and optionally either a fibrinogen level or a thrombin time (TT). In the past, the activated PTT and bleeding time (BT) were recommended as diagnostic tests. However, many patients with VWD have normal PTT and BT results.1

       Initial laboratory evaluation of hemostasis1

  • Complete Blood Count (CBC) and platelet count
  • Partial thromboplastin time (PTT)
  • Prothrombin time (PT)
  • Fibrinogen or thrombin time (TT) (optional)
       Initial VWD assays1
  • VWF:Ag
  • VWF:RCo
  • VWF:FVIII
3_Images_4

 

Indications and Usage

wilate® is a von Willebrand Factor/Coagulation Factor VIII Complex (Human) indicated in children and adults with von Willebrand disease for on-demand treatment and control of bleeding episodes, and for perioperative management of bleeding. wilate® is not indicated for the treatment of hemophilia A.

Important Safety Information

wilate® is contraindicated for patients who have known anaphylactic or severe systemic reaction to plasma-derived products, any ingredient in the formulation, or components of the container.

Hypersensitivity or allergic reactions have been observed upon use of wilate® and may in some cases progress to severe anaphylaxis (including shock) with or without fever.

When using a factor VIII (FVIII)-containing von Willebrand Factor (VWF) product, the treating physician should be aware that continued treatment may cause excessive rise in FVIII activity. Monitor plasma levels of VWF:RCo and FVIII activities in patients receiving wilate® to avoid sustained excessive VWF and FVIII activity levels, which may increase the risk of thrombotic events.

Patients with VWD, especially type 3 patients, may potentially develop neutralizing antibodies (inhibitors) to VWF, manifesting as an inadequate clinical response. Since inhibitor antibodies may occur concomitantly with anaphylactic reactions, patients experiencing an anaphylactic reaction should also be evaluated for the presence of inhibitors.

wilate® is made from human plasma. As with all plasma-derived products, the risk of transmission of infectious agents, eg, viruses and, theoretically, the variant Creutzfeldt-Jakob disease (vCJD) agent or other unknown infectious agents, cannot be completely eliminated. Despite measures to reduce this risk, such products may still potentially transmit disease.

The most common adverse reactions to treatment with wilate® in patients with VWD have been urticaria and dizziness. The most serious adverse reactions to treatment with wilate® in patients with VWD have been hypersensitivity reactions.

Please see full prescribing information.

To report suspected adverse reactions, contact:
Octapharma USA Inc.
866-766-4860 or
FDA at 1-800-FDA-1088 or
www.fda.gov/medwatch

References

1. Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood  Institute (NHLBI) Expert Panel report (USA). Haemophilia. 2008;14:171-232.

2. Sadler JE, Budde U, Eikenboom JCJ, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand   factor. J Thromb Haemost. 2006;4:2103-2114.

3. Mannucci PM. Treatment of von Willebrand’s disease. N Engl J Med. 2004;351:683-694.

Photos are of models and for illustrative purposes only.

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