Replacement therapy for RBDs
| Deficient factor . | Plasma half-life . | Recommended trough levels2 . | On demand dosages . | Recommended trough levels to maintain asymptomatic state after publication of the EN-RBD results24 . |
|---|---|---|---|---|
| Fibrinogen | 2-4 d | 0.5-1 g/L | Cryoprecipitate (15-20 mL/kg) | 1 g/L |
| SD-treated plasma (15-30 mL/kg) | ||||
| Fibrinogen concentrate (50-100 mg/kg) | ||||
| Prothrombin | 3-4 d | 20%-30% | SD-treated plasma (15-25 mL/kg) | >10% |
| FIX concentrate and PCC (20-40 units/kg) | ||||
| Factor V | 36 h | 10%-20% | SD-treated plasma (15-25 mL/kg) | 10% |
| Factor V and factor VIII | FV 36 h | 10%-15% | As for FV | 40% |
| FVIII 10-14 h | ||||
| Factor VII | 4-6 h | 10%-15% | FVII concentrate (30-40 mL/kg) | >20% |
| PCC (20-30 units/kg) | ||||
| rFVIIa (15-30 μg/kg every 4-6 h) | ||||
| Factor X | 40-60 h | 10%-20% | SD-treated plasma (10-20 mL/kg) | >40% |
| PCC (20-30 units/kg) | ||||
| FX/FIX concentrate (10-20 units/kg) | ||||
| Factor XI | 50 h | 15%-20% | SD-treated plasma (15-20 mL/kg) | 15%-20% |
| FXI concentrate (15-20 units/kg) | ||||
| Factor XIII | 9-12 d | 2%-5% | Cryoprecipitate (2-3 bags) | 30% |
| SD-treated plasma (3 mL/kg) | ||||
| FXIII concentrate (until 50 units/kg for high hemorrhagic events) | ||||
| rFXIII-A (35 units/kg) | ||||
| Vitamin K dependent | Vitamin K (10 mg) IV or SC for minor bleeding | No data available | ||
| PCC (20-30 units/kg) with vitamin | ||||
| K (5-20 mg) for severe bleeding or major surgery | ||||
| FFP 15-25 mL/kg is an alternative to PCC |
| Deficient factor . | Plasma half-life . | Recommended trough levels2 . | On demand dosages . | Recommended trough levels to maintain asymptomatic state after publication of the EN-RBD results24 . |
|---|---|---|---|---|
| Fibrinogen | 2-4 d | 0.5-1 g/L | Cryoprecipitate (15-20 mL/kg) | 1 g/L |
| SD-treated plasma (15-30 mL/kg) | ||||
| Fibrinogen concentrate (50-100 mg/kg) | ||||
| Prothrombin | 3-4 d | 20%-30% | SD-treated plasma (15-25 mL/kg) | >10% |
| FIX concentrate and PCC (20-40 units/kg) | ||||
| Factor V | 36 h | 10%-20% | SD-treated plasma (15-25 mL/kg) | 10% |
| Factor V and factor VIII | FV 36 h | 10%-15% | As for FV | 40% |
| FVIII 10-14 h | ||||
| Factor VII | 4-6 h | 10%-15% | FVII concentrate (30-40 mL/kg) | >20% |
| PCC (20-30 units/kg) | ||||
| rFVIIa (15-30 μg/kg every 4-6 h) | ||||
| Factor X | 40-60 h | 10%-20% | SD-treated plasma (10-20 mL/kg) | >40% |
| PCC (20-30 units/kg) | ||||
| FX/FIX concentrate (10-20 units/kg) | ||||
| Factor XI | 50 h | 15%-20% | SD-treated plasma (15-20 mL/kg) | 15%-20% |
| FXI concentrate (15-20 units/kg) | ||||
| Factor XIII | 9-12 d | 2%-5% | Cryoprecipitate (2-3 bags) | 30% |
| SD-treated plasma (3 mL/kg) | ||||
| FXIII concentrate (until 50 units/kg for high hemorrhagic events) | ||||
| rFXIII-A (35 units/kg) | ||||
| Vitamin K dependent | Vitamin K (10 mg) IV or SC for minor bleeding | No data available | ||
| PCC (20-30 units/kg) with vitamin | ||||
| K (5-20 mg) for severe bleeding or major surgery | ||||
| FFP 15-25 mL/kg is an alternative to PCC |
rFVIIa, recombinant activated FVII; SD, solvent/detergent.