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 Half-life 10 to 15 days	Target factor XIII activity: 30 to 50% 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	Target factor XIII activity: 30 to 50% 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	Target factor XIII activity: >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
X Half-life 40 to 70 hours	Target factor X activity: 30 to 50% FX concentrate: 25 international units/kg PCC (3 or 4 factor): 20 to 30 international units/kg FFP: 15 to 20 mL/kg	Target factor X activity: 30 to 50% FX concentrate: 25 international units/kg FFP: 15 to 20 mL/kg	Target factor X activity: >10% FX concentrate: 25 international units/kg FFP: 15 to 20 mL/kg
VII Half-life 4 to 6 hours	Target factor VII activity: 30 to 50% 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	Target factor VII activity: 30 to 50% rFVIIa: 15 to 30 mcg/kg FVII concentrate: 30 to 40 international units/kg FFP: 15 to 20 mL/kg	Target factor VII activity: >10% FVII concentrate: 30 to 40 international units/kg FFP: 15 to 20 mL/kg
V Half-life 16 to 36 hours	Target factor V activity: 30 to 50% FFP: 15 to 20 mL/kg	Target factor V activity: 30 to 50% FFP: 15 to 20 mL/kg	Target factor V activity: >5% FFP: 15 to 20 mL/kg
II (prothrombin) Half-life 3 to 4 days	Target factor II activity: 30 to 50% PCC (3 or 4 factor): 20 to 30 international units/kg FFP: 15 to 20 mL/kg	Target factor II activity: 30 to 50% FFP: 15 to 20 mL/kg	Target factor II activity: >10% 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, Hemophilia Treatment Center (HTC), or 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 and rFVIIa generally are not used for treating 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.

FFP: Fresh Frozen Plasma; PCC: prothrombin complex concentrate; rFVIIa: recombinant activated factor VII; rFXIII A-subunit: recombinant factor XIII A-subunit; RICDs: rare inherited coagulation disorders.

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

Data on half-lives and target factor activity levels derived from: Peyvandi F, Palla R, Menegatti M et al. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders. J Thromb Haemost 2012; 10:615.

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