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To correct VWF deficit without excessively increasing FVIII:C plasma levels†, a high VWF:FVIII ratio has been recommended.2,3
Appropriate determination of FVIII levels is advised to guide the dose to be administered and the frequency of repeated infusions in order to avoid an excessive rise in FVIII levels.1
Voncento has a VWF:FVIII ratio of 2.4:1.
†Normal FVIII plasma level range is 50-150%.4
Available in two vial sizes1 | |||
---|---|---|---|
Voncento | IU VWF | IU FVIII | Solution after reconstitution (IU VWF per ml) |
5ml | 1200 | 500 | 240 |
10ml | 2400 | 1000 | 240 |
Calculate the dose using the number of IU of VWF:RCo specified.
Usually 40 - 80 IU/kg of Von Willebrand factor (VWF:RCo) corresponding to 20 - 40 IU FVIII:C/kg of body weight are recommended to achieve haemostasis.
An initial dose of 80 IU/kg VWF:RCo may be required in adults, especially in patients with type 3 VWD where maintenance of adequate levels may require greater doses than in other types of VWD.
The dose and frequency of administration should always be oriented to the clinical effectiveness in the individual case.
8 years old, weighs 20kg and has type 2 VWD
Prescribed 40 IU/kg VWF:RCo twice weekly (equating to 800 units VWF:RCo twice weekly) for laceration to left leg requiring sutures
*Calculations use maximum infusion rate (6ml/min) from the Voncento SPC1
3.3ml in 0.6min*
For long term prophylaxis, a dose of 25 - 40 IU VWF:RCo/kg body weight should be considered at a frequency of 1 to 3 times per week.
In patients with gastrointestinal bleeds or menorrhagia, shorter dose intervals or higher doses may be necessary.
The dose and duration of treatment will depend on the clinical status of the patient, as well as their VWF:RCo and FVIII:C plasma levels.
26 years old, weighs 65kg and has type 3 VWD
Prescribed 25 IU/kg VWF:RCo twice weekly (equating to 1625 units VWF:RCo twice weekly) with a history of menorrhagia
*Calculations use maximum infusion rate (6ml/min) from the Voncento SPC1
6.8ml in 1.1min*
For long term prophylaxis, a dose of 40 - 80 IU VWF:RCo/kg body weight one to three times a week should be considered.
The dose and duration of treatment will depend on the clinical status of the patient, as well as their VWF:RCo and FVIII:C plasma levels.
10 years old, weighs 30kg and has type 3 VWD
Prescribed 40 IU/kg VWF:RCo once weekly (equating to 1200 units VWF:RCo once weekly) with a history of GI bleeding
*Calculations use maximum infusion rate (6ml/min) from the Voncento SPC1
5.0ml in 0.8min*
For prevention of excessive bleeding during or after surgery the application should start 1-2 hours before surgical procedure.
An appropriate dose should be re-administered every 12-24 hours.
The dose and duration of the treatment depend on the clinical status of the patient, the type and severity of the bleeding and both VWF:RCo and FVIII:C levels.
‡Do not store above 25°C
References
1. CSL Berhing UK Limited. Voncento Summary of Product Characteristics. 2. Franchini M, Targher G, et al. Antithrombotic prophylaxis in patients with Von Willebrand disease undergoing major surgery: when is it necessary? J Thromb Thrombolysis. 2009;28(2):215-219. 3. Rodeghiero F, Castaman G, Tosetto A. Optimizing treatment of Von Willebrand disease by using phenotypic and molecular data. American Society of Hematology. 2009;113-123. 4. National Hemophilia Foundation. Hemophilia A (Factor VIII Deficiency). https://www.hemophilia.org/bleeding-disorders-a-z/types/hemophilia-a. Accessed March 2022.
Adverse events should be reported. Reporting forms and information can be found at https://yellowcard.mhra.gov.uk. Adverse events should also be reported to CSL Behring UK Ltd. on 01444 447 405.