Haemophilia A with factor VIII inhibitors, severe haemophilia A

Active Ingredient: Emicizumab

Indication for Emicizumab

Population group: only infants (40 days - 1 year old) , children (1 year - 12 years old) , adolescents (12 years - 18 years old) , adults (18 years old or older)

Emicizumab is indicated for routine prophylaxis of bleeding episodes in patients with:

  • haemophilia A (congenital factor VIII deficiency) with factor VIII inhibitors
  • severe haemophilia A (congenital factor VIII deficiency, FVIII <1%) without factor VIII inhibitors.

For this indication, competent medicine agencies globally authorize below treatments:

1.5-3 mg/kg once a week

Route of admnistration

Subcutaneous

Defined daily dose

1.5 - 3 mg per kg of body weight

Dosage regimen

From 1.5 To 3 mg per kg of body weight once every 7 day(s)

Loading dose

3 mg per kg of body weight

Maintenance dose

1.5 mg per kg of body weight

Detailed description

Treatment (including routine prophylaxis) with bypassing agents (e.g. aPCC and rFVIIa) should be discontinued the day before starting emicizumab therapy.

Factor VIII (FVIII) prophylaxis may be continued for the first 7 days of emicizumab treatment.

The recommended dose is 3 mg/kg once weekly for the first 4 weeks (loading dose), followed by maintenance dose of either 1.5 mg/kg once weekly, 3 mg/kg every two weeks, or 6 mg/kg every four weeks, all doses administered as a subcutaneous injection.

Delayed or missed doses

If a patient misses a scheduled subcutaneous injection of Hemlibra, the patient should be instructed to take the missed dose as soon as possible, up to a day before the day of the next scheduled dose. The patient should then administer the next dose on the usual scheduled dosing day. The patient should not take two doses on the same day to make up for a missed dose.

Dosage considerations

The injection should be restricted to the recommended injection sites: the abdomen, the upper outer arms and the thighs.

Active ingredient

Emicizumab

Emicizumab is a humanized monoclonal modified immunoglobulin G4 (IgG4) antibody with a bispecific antibody structure. Emicizumab bridges activated factor IX and factor X to restore the function of missing activated factor VIII that is needed for effective haemostasis.

Read more about Emicizumab

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