Haemophilia B/Factor IX Deficiency (With No Inhibitors)
Prophylaxis Treatment on demand ICD-10 code: D67 PROPHYLAXIS Prophylaxis (primary and secondary) should be considered for patients with severe haemophilia B (<1% factor activity) who can access the health facility twice weekly for infusions; or have indwelling venous catheters and are candidates for home-based care. Primary prophylaxis: Prophylaxis started in the absence of documented joi
ICD-10 code: D67
PROPHYLAXIS
Prophylaxis (primary and secondary) should be considered for patients with severe haemophilia B (<1% factor activity) who can access the health facility twice weekly for infusions; or have indwelling venous catheters and are candidates for home-based care.
| Primary prophylaxis: | Prophylaxis started in the absence of documented joint disease/damage. |
| Secondary prophylaxis: | Prophylaxis initiated after joint damage has occurred |
- Factor IX, intravenous 25 units/kg, twice weekly.
TREATMENT ON DEMAND
Minor bleeds
Bleeds into the muscle or soft tissue, mouth or gums, epistaxis, painless haematuria and early joint bleeds.
- Factor IX/factor IX complex, intravenous, 50 units/kg immediately as a single dose.
- If there is evidence of ongoing bleeding after 12 hours, consult the local Haemophilia Treatment Centre.
Major bleeds
Major muscle or joint bleeds result from severe injury or bleeds that affect the central nervous system, gastrointestinal system, neck or throat, hip or iliopsoas muscle, or forearm compartment.
- Factor IX/factor IX complex, intravenous, 50-80 units/kg.
- All of these patients need hospitalisation.
Discuss all patients promptly with the local haemophilia treatment Centre to plan ongoing treatment and factor replacement.
Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_ Updated February 2025
Chapter 3: Blood and Blood Forming Organs, 3.6 Haemophilia A and B, 3.6.2 Haemophilia B/Factor IX Deficiency (With No Inhibitors)