Table 4

Replacement therapy for RBDs

Deficient factorPlasma half-lifeRecommended trough levelsOn demand dosagesRecommended 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 factorPlasma half-lifeRecommended trough levelsOn demand dosagesRecommended 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.

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