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Recombinant factor VIIa in an infant with haemophilia A and inhibitors
L Tengborn1, B Kjellman, P O Elfstrand
1Section of Coagulation Disorders, Sahlgren's Hospital, Göteborg, Sweden.
Insights
Severe hemophilia A bleeding in a child was treated successfully with recombinant factor VIIa after standard treatments failed due to factor VIII antibodies. This approach effectively controlled hemorrhage without complications.
Area of Science:
- Hematology
- Pediatric Medicine
- Coagulation Disorders
Background:
- Severe hemophilia A is a rare inherited bleeding disorder.
- Factor VIII concentrate and tranexamic acid are standard treatments.
- Development of inhibitors (antibodies) to factor VIII complicates management.
Observation:
- A 16-month-old boy with severe hemophilia A experienced persistent gingival bleeding post-trauma.
- Standard treatment with factor VIII concentrate and tranexamic acid was ineffective.
- Inhibitors to factor VIII were detected in the patient.
Findings:
- Treatment with recombinant factor VIIa at 60-90 micrograms/kg body weight, administered four to eight times daily, was initiated.
- Combined with local hemostatic measures, recombinant factor VIIa successfully controlled the gingival bleeding.
- No adverse events or complications were observed during or after treatment.
Implications:
- Recombinant factor VIIa is an effective alternative treatment for hemophilia A patients with high-titer inhibitors.
- This case highlights the importance of monitoring for inhibitor development in hemophilia patients.
- Successful management of bleeding episodes in inhibitor-positive hemophilia patients is achievable with advanced therapies.
Abstract:
Post-traumatic bleeding from the gingiva in a 16-month-old boy with severe haemophilia A did not stop after treatment with factor VIII concentrate and tranexamic acid, and it was demonstrated that he had developed antibodies to factor VIII. Treatment with recombinant factor VIIa 60-90 micrograms/kg body weight four to eight times daily, together with local measures, stopped the bleeding and no complications were seen.