Table 2.
Suggested dosing regimens of bypass agents in hemophilia patients with inhibitors
| Agent | Dosing for minor surgery | Dosing for major surgery | Comments |
|---|---|---|---|
| rFVIIa | 90 µg/kg/dose immediately before surgery and every 2 hours for 2 days and then every 2-6 hours until healed. | 90 µg/kg/dose immediately before surgery and every 2 hours for 2 days. Then every 2-3 hours for 5 days, then every 4 hours until days 7-10, then every 6 hours until days 14-21 | • Preferred agent for patients with HB and high-titer inhibitors per WFH guidelines since aPCC contains FIX and may cause or worsen an allergic reaction. • For patients with FIX deficiency, low dose (15-30 µg/kg) in combination with TXA has been shown to be effective for major surgery in limited studies and can be considered for those at risk of thrombosis.33 |
| Activated prothrombin concentrate complex | 50-100 U/kg immediately before surgery and then every 6-12 hours | 50-100 U/kg immediately before surgery, then every 6-12 hours | • Maximum: 100 units/kg/dose; 200 units/kg/d). • Less preferred for HB patients with high-titer inhibitors per WFH guidelines. |