Table 5

General recommendations for long-term prophylaxis

Deficient factorRecommended trough levelsReported dose schedule for successful long-term prophylaxisNotes
ProductsDoseFrequency
Fibrinogen0.5-1 g/LCryoprecipitate1 unit3 times/wkAfibrinogenemic patients with recurrent life-threatening bleedings or undergoing surgeries
3 unitsEvery 7-10 d
Fibrinogen concentrate30-100 mg/kgEvery wk
Prothrombin20%-30%PCC20-40 units/kg1 time/wk
Factor V10%-20%SD-treated plasma20-30 mL/kg2 times/wk
Factor V and factor VIII10%-15%No data
Factor VII10%-15%SD-treated plasma10-15 mL/kg2 times/wkPrevention of bleeding during surgery or in children with recurrent hemarthrosis or CNS
pdFVII10-40 units/kg3 times/wk
rFVIIa20-40 µg/kg2-3 times/wk
Factor X10%-20%PCC20-40 units/kg2-3 times/wkPatients with recurrent life-threatening bleedings or undergoing surgeries
FX/FIX concentrate20-40 units/kg1-2 times/wk
Factor XI15%-20%No data
Factor XIII2%-5%Cryoprecipitate2 unitsEvery 3 wkHighly recommended in severe patients
SD-treated plasma15-20 mL/kgEvery 4-6 wk
FXIII concentrate10-40 units/kgEvery 4-6 wk
rFXIII-A35 units/kgEvery 4
Vitamin K dependentVitamin K5-20 mg1 time/wk orally
  • pdFVII, plasma-derived FVII; rFVIIa, recombinant activated FVII; SD, solvent/detergent.