Hemophilia A
One IU of Factor VIII (FVIII) activity per kg body weight will increase the circulating FVIII level by approximately 2.0 International Units (IU)/dL. Dosage must be individualized based on the patient's weight, type and severity of hemorrhage, FVIII level, and presence of inhibitors. Judge the adequacy of treatment by clinical effects and, in all cases, adjust doses as needed based on clinical judgment and on frequent monitoring of the patient's FVIII level. Table 1 provides dosing recommendations for the treatment of hemophilia A in adults.
Table 1: Dosing Recommendations for the Treatment of Hemophilia A in Adults1 Hemorrhagic Event Dosage (IU FVIII:C/kg Body Weight)
IU = International Units.
Minor hemorrhage:
Early joint or muscle bleed
Severe epistaxis
Loading dose 15 IU FVIII:C/kg to achieve a FVIII:C plasma level of approximately 30% of normal; one infusion may be sufficient. If needed, half of the loading dose may be given once or twice daily for 1-2 days.
Moderate hemorrhage:
Advanced joint or muscle bleed
Neck, tongue, or pharyngeal hematoma (without airway compromise)
Tooth extraction
Severe abdominal pain
Loading dose 25 IU FVIII:C/kg to achieve a FVIII:C plasma level of approximately 50% of normal, followed by 15 IU FVIII:C/kg every 8-12 hours for the first 1-2 days to maintain the FVIII:C plasma level at 30% of normal.
Continue the same dose once or twice daily for up to 7 days or until adequate wound healing is achieved.
Life-threatening hemorrhage:
Major surgery
Gastrointestinal bleeding
Neck, tongue, or pharyngeal hematoma (with potential for airway compromise)
Intracranial, intraabdominal, or intrathoracic bleeding
Fractures
Initially 40-50 IU FVIII:C/kg, followed by 20-25 IU FVIII:C/kg every 8 hours to maintain the FVIII:C plasma level at 80-100% of normal for 7 days.
Continue the same dose once or twice daily for another 7 days to maintain the FVIII:C level at 30-50% of normal.
2.2Treatment of Bleeding Episodes in VWD
Administer 40 to 80 International Units (IU) VWF:RCo (corresponding to 17 to 33 International Units (IU) FVIII in Humate-P) per kg body weight every 8 to 12 hours. Adjust the dosage based on the extent and location of bleeding. Administer repeat doses as long as needed based on monitoring of appropriate clinical and laboratory measures (see Warnings and Precautions [5.2 and 5.3]). Expected levels of VWF:RCo are based on an expected in vivo recovery (IVR) of 2.0 International Units (IU)/dL rise per International Unit (IU)/kg VWF:RCo administered. The administration of 1 International Unit (IU) of FVIII per kg body weight can be expected to lead to a rise in circulating VWF:RCo of approximately 5 International Units (IU)/dL. Table 2 provides dosing recommendations for adult and pediatric patients (see also Pediatric Use [8.4]).2
Table 2: VWF:RCo Dosing Recommendations for the Treatment of Bleeding Episodes by VWD Type VWD Type Severity of Hemorrhage Dosage (IU* VWF:RCo/kg Body Weight)
*
IU = International Units.
†
For major bleeds in all types of VWD where repeated dosing is required, monitor and maintain the patient's FVIII level according to the guidelines for hemophilia A therapy.
Type 1 VWD – Mild
(baseline VWF:RCo activity typically >30%) Minor
(e.g., epistaxis, oral bleeding, menorrhagia) Typically treatable with desmopressin.
Minor
(when desmopressin i |
