From the FDA Drug Label
Desmopressin acetate injection 4 mcg/mL is indicated for patients with mild to moderate classic von Willebrand’s disease (Type I) with factor VIII levels greater than 5% Dose-response studies were performed in healthy persons, using doses of 0.1 to 0.4 mcg/kg body weight, infused over a 10-minute period. Maximal dose response occurred at 0.3 to 0. 4 mcg/kg.
The appropriate dosing for Von Willebrand Factor (VWF) using desmopressin is 0.3 mcg/kg infused over a 10-minute period, as this dose has been shown to produce a maximal response in increasing plasma levels of factor VIII activity in patients with von Willebrand’s disease Type I 1 1.
From the Research
Von Willebrand factor (VWF) replacement therapy should be dosed at 50-80 IU/kg for major bleeding or surgical procedures, with dosing individualized based on the patient's baseline VWF levels, type of VWD, severity of bleeding, and clinical response, as supported by the most recent study 2.
Overview of VWF Replacement Therapy
VWF replacement therapy is used to treat patients with von Willebrand disease (VWD), a bleeding disorder caused by a deficiency or defect of VWF. Treatment options include desmopressin (DDAVP) for milder cases and VWF concentrates for more severe cases or when DDAVP is contraindicated.
Dosing Considerations
- For minor bleeding episodes, a dose of 40-60 IU/kg may be sufficient, while major bleeding or surgery may require dosing every 12-24 hours for 3-10 days.
- The dosing should be individualized based on the patient's baseline VWF levels, type of VWD, severity of bleeding, and clinical response.
- Monitoring therapy through VWF activity levels (ristocetin cofactor activity) and factor VIII levels is important to ensure adequate hemostasis, with target levels typically 50-100% for minor bleeding and >100% for major bleeding or surgery.
Recent Study Findings
A recent study 2 found that peri-procedural DDAVP prophylaxis appears to be effective among individuals with VWD, with excessive bleeding occurring in only 4/31 major procedures and 2/55 minor procedures. Another study 3 quantified the relationship between desmopressin concentration and VWF, finding that VWF activity increased 452% with an EC50 of 0.174 ng/ml.
Clinical Implications
The findings of these studies support the use of VWF replacement therapy in patients with VWD, with individualized dosing based on the patient's specific needs. The most recent and highest quality study 2 should be prioritized when making treatment decisions, as it provides the most up-to-date and reliable evidence on the effectiveness of VWF replacement therapy.
Key Points
- VWF replacement therapy should be dosed at 50-80 IU/kg for major bleeding or surgical procedures.
- Dosing should be individualized based on the patient's baseline VWF levels, type of VWD, severity of bleeding, and clinical response.
- Monitoring therapy through VWF activity levels and factor VIII levels is important to ensure adequate hemostasis.
- Recent studies support the use of VWF replacement therapy in patients with VWD, with individualized dosing based on the patient's specific needs.