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.