Table 2

Doses of factor VIII in factor VIII-VWF concentrates that are recommended in VWD patients who are unresponsive to desmopressin

Type of bleedingFVIII:C dose,* U/kgNo. of infusionsObjective
Major surgery 50 Once a day or every other day Maintain FVIII:C 80-100 IU/dL for the first 2 days and then > 50 IU/dL for 5-7 days 
Minor surgery; cesarean section 30 Once a day or every other day Maintain FVIII:C > 30 IU/dL for at least 5-7 days 
Dental extractions 20-40 Single Obtain FVIII:C >30-50 IU/dL; subsequent infusions on clinical need 
Delivery and/or epidural anesthesia 30-40 Single Obtain FVIII:C > 50 IU/dL; subsequent infusions on clinical need 
Spontaneous or posttraumatic bleeding 20-40 Single Obtain FVIII:C > 30-50 IU/dL; subsequent infusions on clinical need 
Severe frequent bleeding (gastrointestinal, menorrhagia, hemarthrosis) 20-40 Twice to thrice weekly Short-term control: dose adjusted on the basis of clinical response; life-long prophylaxis: still experimental, to be adopted within clinical trials 
Type of bleedingFVIII:C dose,* U/kgNo. of infusionsObjective
Major surgery 50 Once a day or every other day Maintain FVIII:C 80-100 IU/dL for the first 2 days and then > 50 IU/dL for 5-7 days 
Minor surgery; cesarean section 30 Once a day or every other day Maintain FVIII:C > 30 IU/dL for at least 5-7 days 
Dental extractions 20-40 Single Obtain FVIII:C >30-50 IU/dL; subsequent infusions on clinical need 
Delivery and/or epidural anesthesia 30-40 Single Obtain FVIII:C > 50 IU/dL; subsequent infusions on clinical need 
Spontaneous or posttraumatic bleeding 20-40 Single Obtain FVIII:C > 30-50 IU/dL; subsequent infusions on clinical need 
Severe frequent bleeding (gastrointestinal, menorrhagia, hemarthrosis) 20-40 Twice to thrice weekly Short-term control: dose adjusted on the basis of clinical response; life-long prophylaxis: still experimental, to be adopted within clinical trials 
*

It is preferable that FVIII/VWF concentrates are labeled for both FVIII:C and VWF:RCo content and with VWF/FVIII ≥ 1.

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