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Prophylaxis in severe haemophilia B
Adult: Human plasma-derived: 20-40 IU/kg every 3-4 days as required. Recombinant: As nonacog alfa: 40 IU/kg (range 13 to 78 IU/kg) every 3-4 days. As nonacog gamma: 40-60 IU/kg every 3-4 days, may be titrated based on age, bleeding pattern, or physical activity. As albutrepenonacog alfa: 35-50 IU/kg once wkly, or up to 75 IU/kg every 10 or 14 days in patients w/ well-controlled conditions. As eftrenonacog alfa: 50 IU/kg once wkly or 100 IU/kg once every 10 days, adjust based on individual response.
Child: Recombinant: As nonacog alfa: 63.7 (±19.1) IU/kg every 3 to 7 days. Shorter intervals or higher doses may be necessary in younger patients. As nonacog gamma: <12 yr 40-80 IU/kg every 3-4 days; ≥12 yr Same as adult dose. Doses may be titrated based on age, bleeding pattern, or physical activity. As albutrepenonacog alfa: 35-50 IU/kg once wkly. As eftrenonacog alfa: <12 yr 50-60 IU/kg once wkly; ≥12 yr Same as adult dose. Max: 100 IU/kg.
Child: Recombinant: As nonacog alfa: 63.7 (±19.1) IU/kg every 3 to 7 days. Shorter intervals or higher doses may be necessary in younger patients. As nonacog gamma: <12 yr 40-80 IU/kg every 3-4 days; ≥12 yr Same as adult dose. Doses may be titrated based on age, bleeding pattern, or physical activity. As albutrepenonacog alfa: 35-50 IU/kg once wkly. As eftrenonacog alfa: <12 yr 50-60 IU/kg once wkly; ≥12 yr Same as adult dose. Max: 100 IU/kg.
Intravenous
Replacement therapy in haemophilia B
Adult: Human plasma-derived or recombinant: Dosage and duration is individualised depending on the preparation used, severity of the disease, and type of surgery to be performed. Suggested target factor IX concentrations: Mild to moderate haemorrhage: Increase to 20-40% of normal. More serious haemorrhage or minor surgery: Increase to 30-60% of normal. Severe haemorrhage or major surgery: Increase to 60-100% of normal.
