Factor replacement products for bleeding or surgery in patients with rare inherited coagulation factor deficiencies

Deficient factor	Major bleeding/surgery (treat until healing is complete)	Minor bleeding/surgery (treat for 2 to 3 days)	Prophylaxis
XIII Target level >5%	rFXIII A-subunit: 35 international units/kg FXIII concentrate: 10 to 20 international units/kg FFP: 15 to 20 mL/kg Cryoprecipitate: 1 unit/10 kg	rFXIII A-subunit: 35 international units/kg FXIII concentrate: 10 to 20 international units/kg FFP: 15 to 20 mL/kg Cryoprecipitate: 1 unit/10 kg	rFXIII A-subunit: 35 international units/kg FXIII concentrate: 10 to 20 international units/kg FFP: 15 to 20 mL/kg Cryoprecipitate: 1 unit/10 kg
X Target level >20%	FX concentrate: 25 international units/kg PCC (3 or 4 factor): 20 to 30 international units/kg FFP: 15 to 20 mL/kg	FX concentrate: 25 international units/kg FFP: 15 to 20 mL/kg	FX concentrate: 25 international units/kg PCC (3 or 4 factor) FFP: 15 to 20 mL/kg
VII Target level >20%	rFVIIa: 15 to 30 mcg/kg FVII concentrate: 30 to 40 international units/kg PCC (4 factor): 20 to 30 international units/kg FFP: 15 to 20 mL/kg	rFVIIa: 15 to 30 mcg/kg FVII concentrate: 30 to 40 international units/kg FFP: 15 to 20 mL/kg	rFVIIa FVII concentrate: 30 to 40 international units/kg
V Target level >20%	FFP: 15 to 20 mL/kg	FFP: 15 to 20 mL/kg	FFP: 15 to 20 mL/kg
II Target level >30%	PCC (3 or 4 factor): 20 to 30 international units/kg FFP: 15 to 20 mL/kg	FFP: 15 to 20 mL/kg	FFP: 15 to 20 mL/kg

This table serves as a general guide but does not substitute for clinical judgment in the management of bleeding; early involvement of a hematologist or a hemophilia treatment center or a clinician with expertise in managing coagulation factor deficiencies is advised. Dose adjustments may be required depending on severity of bleeding and factor activity levels. PCCs generally are not used in cases of minor bleeding or minor surgery due to their potential prothrombotic risks. Refer to UpToDate topics on rare coagulation disorders for further details including dosing intervals, indications for prophylaxis, other potential therapies, potential risks and complications of therapy, and other aspects of management. Refer to UpToDate topics on hemophilia and factor XI deficiency for therapies used for factor VIII, IX, and XI deficiencies.

RICDs: rare inherited coagulation disorders; rFXIII A-subunit: recombinant factor XIII A-subunit; FFP: fresh frozen plasma; PCC: prothrombin complex concentrate; rFVIIa: recombinant activated factor VII.

Adapted from: Mannucci PM, Duga S, Peyvandi F. Recessively inherited coagulation disorders. Blood 2004; 104:1243.

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