General recommendations for long-term prophylaxis
| Deficient factor . | Recommended trough levels . | Reported dose schedule for successful long-term prophylaxis . | Notes . | ||
|---|---|---|---|---|---|
| Products . | Dose . | Frequency . | |||
| Fibrinogen | 0.5-1 g/L | Cryoprecipitate | 1 unit | 3 times/wk | Afibrinogenemic patients with recurrent life-threatening bleedings or undergoing surgeries |
| 3 units | Every 7-10 d | ||||
| Fibrinogen concentrate | 30-100 mg/kg | Every wk | |||
| Prothrombin | 20%-30% | PCC | 20-40 units/kg | 1 time/wk | — |
| Factor V | 10%-20% | SD-treated plasma | 20-30 mL/kg | 2 times/wk | — |
| Factor V and factor VIII | 10%-15% | No data | — | ||
| Factor VII | 10%-15% | SD-treated plasma | 10-15 mL/kg | 2 times/wk | Prevention of bleeding during surgery or in children with recurrent hemarthrosis or CNS |
| pdFVII | 10-40 units/kg | 3 times/wk | |||
| rFVIIa | 20-40 µg/kg | 2-3 times/wk | |||
| Factor X | 10%-20% | PCC | 20-40 units/kg | 2-3 times/wk | Patients with recurrent life-threatening bleedings or undergoing surgeries |
| FX/FIX concentrate | 20-40 units/kg | 1-2 times/wk | |||
| Factor XI | 15%-20% | No data | — | ||
| Factor XIII | 2%-5% | Cryoprecipitate | 2 units | Every 3 wk | Highly recommended in severe patients |
| SD-treated plasma | 15-20 mL/kg | Every 4-6 wk | |||
| FXIII concentrate | 10-40 units/kg | Every 4-6 wk | |||
| rFXIII-A | 35 units/kg | Every 4 | |||
| Vitamin K dependent | Vitamin K | 5-20 mg | 1 time/wk orally | — | |
| Deficient factor . | Recommended trough levels . | Reported dose schedule for successful long-term prophylaxis . | Notes . | ||
|---|---|---|---|---|---|
| Products . | Dose . | Frequency . | |||
| Fibrinogen | 0.5-1 g/L | Cryoprecipitate | 1 unit | 3 times/wk | Afibrinogenemic patients with recurrent life-threatening bleedings or undergoing surgeries |
| 3 units | Every 7-10 d | ||||
| Fibrinogen concentrate | 30-100 mg/kg | Every wk | |||
| Prothrombin | 20%-30% | PCC | 20-40 units/kg | 1 time/wk | — |
| Factor V | 10%-20% | SD-treated plasma | 20-30 mL/kg | 2 times/wk | — |
| Factor V and factor VIII | 10%-15% | No data | — | ||
| Factor VII | 10%-15% | SD-treated plasma | 10-15 mL/kg | 2 times/wk | Prevention of bleeding during surgery or in children with recurrent hemarthrosis or CNS |
| pdFVII | 10-40 units/kg | 3 times/wk | |||
| rFVIIa | 20-40 µg/kg | 2-3 times/wk | |||
| Factor X | 10%-20% | PCC | 20-40 units/kg | 2-3 times/wk | Patients with recurrent life-threatening bleedings or undergoing surgeries |
| FX/FIX concentrate | 20-40 units/kg | 1-2 times/wk | |||
| Factor XI | 15%-20% | No data | — | ||
| Factor XIII | 2%-5% | Cryoprecipitate | 2 units | Every 3 wk | Highly recommended in severe patients |
| SD-treated plasma | 15-20 mL/kg | Every 4-6 wk | |||
| FXIII concentrate | 10-40 units/kg | Every 4-6 wk | |||
| rFXIII-A | 35 units/kg | Every 4 | |||
| Vitamin K dependent | Vitamin K | 5-20 mg | 1 time/wk orally | — | |
pdFVII, plasma-derived FVII; rFVIIa, recombinant activated FVII; SD, solvent/detergent.