Humate-P Dosing for Von Willebrand Disease
For bleeding episodes and surgical prophylaxis in von Willebrand disease, administer Humate-P at 40-80 IU/kg based on VWF:RCo activity, with loading doses typically 60-80 IU/kg for major surgery and 40-60 IU/kg for minor bleeding, followed by maintenance doses of 40-60 IU/kg every 8-24 hours depending on clinical response. 1, 2, 3
Dosing by Clinical Indication
Surgical Prophylaxis
- Major surgery: Loading dose of 60-80 IU VWF:RCo/kg, followed by maintenance doses of 40-60 IU/kg every 12-24 hours 3, 4
- Minor surgery (including oral procedures): Loading dose of 40-60 IU VWF:RCo/kg 4
- Median loading doses in clinical trials ranged from 42.6 IU/kg (oral surgery) to 82.3 IU/kg (urgent surgery) 3, 4
- Continue treatment for median of 3-10 days post-operatively, with most surgical events requiring ≤10 days of therapy 5
Acute Bleeding Episodes
- Initial dose: 40-80 IU VWF:RCo/kg depending on bleeding severity 2, 5
- Median dose per infusion for bleeding events: 55.3 IU VWF:RCo/kg (range 17.1-227.5) 5
- Repeat dosing every 8-24 hours based on clinical response and laboratory monitoring 2, 6
Prophylactic Therapy
- Maintenance prophylaxis: 40-50 IU VWF:RCo/kg administered 2-3 times weekly 5
- Median prophylactic dose: 41.6 IU VWF:RCo/kg (range 34.6-81.0) 5
Special Populations
Pediatric Dosing
- Use same weight-based dosing as adults (40-80 IU/kg based on indication) 5, 4
- Clinical trials demonstrated 95-100% excellent/good efficacy across all pediatric age groups (infants, children, adolescents) 5
- Pharmacokinetic assessments should guide individualized dosing in children undergoing surgery 4
ECMO-Associated Acquired von Willebrand Syndrome
- Pediatric patients: 15-30 IU/kg once, with additional doses for persistent bleeding 1
- Adult patients: 40 IU/kg once, which significantly increases VWF levels within 2 hours 1
- Important caveat: Risk of thrombotic complications (30% in one study), including LVAD thrombosis and DVT 1
Pharmacokinetic Considerations
- VWF:RCo half-life: Median 11.7 hours 4
- Incremental recovery: Median 2.4 IU/dL per IU/kg infused 4
- VWF:RCo to FVIII:C ratio: Approximately 2.4:1 in Humate-P 2, 6
- Dosing should be based on VWF:RCo activity rather than FVIII:C content 3, 5, 6
Monitoring and Dose Adjustment
- Measure VWF:RCo and FVIII:C levels before surgery and periodically during treatment 3, 4
- Target VWF:RCo levels: 50-100 IU/dL for minor procedures, 100 IU/dL for major surgery 3, 4
- Adjust subsequent doses based on laboratory values and clinical bleeding response 2, 6
- Monitor for thrombotic complications, particularly in ECMO patients or those receiving prolonged high-dose therapy 1
Safety Profile
- Adverse events are rare, with only 4% of patients experiencing treatment-related effects in large studies 5
- No serious adverse events or documented viral transmission in nearly 30 years of clinical use 2, 6
- Thrombotic risk is minimal in standard VWD treatment but elevated in ECMO-associated AVWS 1, 2
- Common mild adverse events include peripheral edema and extremity pain 3