Which medications in the drug class Blood Product Derivatives are used in the treatment of Hemophilia C?

Updated: Dec 18, 2020
  • Author: Vineeta Gupta, MD, DNB, MAMS, FRCPCH, FUICC; Chief Editor: Hassan M Yaish, MD  more...
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Blood Product Derivatives

FFP is the first product used to treat patients with hemophilia C. The main advantage of FFP is its availability. Disadvantages to its use include the large volumes required, the potential for transmission of infective agents, and the possibility of allergic reactions.

Solvent-detergent–treated FFP has a factor XI half-life similar to FFP (mean, 45 h). It is safer than regular FFP, because it reduces the transmission of known enveloped viral agents. However, it is not protective against nonenveloped viruses, such as hepatitis A virus.

Fresh frozen plasma

This is the product of choice when factor XI concentrates are not available. FFP is easily available. It can be infused over a short period. Disadvantages include large infusion volumes to achieve appropriate control of bleeding, a potential for transmitting infective agents, and the possibility of allergic reactions.

Human coagulation factor Xl

Factor XI concentrates provide the best source for factor XI replacement. Two products available in Europe are Hemoleven (Laboratoire français du Fractionnement et des Biotechnologies [LFB], Les Ulis, France) and factor XI concentrate (Bio Products Laboratory [BPL], Elstree, Hertfordshire, United Kingdom). Both are heat treated and are not expected to transmit the human immunodeficiency virus (HIV) or hepatitis viruses. Both products also contain antithrombin III and heparin in different concentrations. These products appear to provide good treatment for selected patients.

The typical dose is 30 U/kg or less.

Advantages of factor XI concentrates include selective delivery of the deficient factor, a reduced volume of infusion, and viral safety. However, they are plasma-derived products; therefore, they can have all of the attendant disadvantages of any plasma-derived product.

Several issues are encountered with the use of factor XI concentrates. The 2 previously mentioned concentrates, Hemoleven (LFB) and factor XI concentrate (BPL), are hemostatically effective and virally safe but are associated with evidence of activation of the coagulation system and some thrombotic events, especially in patients with preexisting vascular disease.

Briggs et al studied 229 treatment episodes with factor XI concentrate (BPL) in 161 patients aged 3-88 years and observed 21 adverse events in 19 patients, 12 of whom were probably or definitely thrombotic. Good hemostatic efficacy was reported in all. No transmission of HIV or hepatitis was reported. The mean factor XI recovery was 91% of the injected dose, and the mean half-life was 52 hours.

The BPL Factor XI was also used in the United States in a study of elective surgery in 12 patients aged 24-81 years. Only 1 patient developed anaphylaxis, and laboratory (not clinical) evidence of disseminated intravascular coagulation (DIC) was present. In all other patients, BPL was used successfully. Hemoleven, (LFB) produced results similar to those of factor XI concentrate from BPL. Thirty-one patients, aged 5-76 years, undergoing 33 procedures received Hemoleven (LFB). Recovery was 80%, with a half-life of 46 hours (range, 32-52 h). Thromboembolic events occurred as complication in 3 infusions exceeding 30 U/kg.

Hemoleven and factor XI concentrate (BPL) are heat treated and are therefore not expected to transmit HIV or hepatitis viruses.

Fibrin sealant (Tisseel, Artiss, Evicel)

Fibrin glue is sometimes used as an adjunct to or substitute for plasma products. Centeon glue (Beriplast) has been used successfully in Israel in patients with congenital bleeding disorders who are undergoing dental extractions without blood product replacement. The glue is applied with a pair of syringes, one containing calcium and thrombin and one containing fibrinogen, factor XIII, and aprotinin.

The US Food and Drug Administration (FDA) approved a fibrin sealant (Tisseel VH; Baxter Healthcare Corporation, Westlake Village, Calif) for adjunctive topical hemostasis. Fibrin sealant contains fibrinogen (sealer protein) as the main active ingredient and fibrinolysis inhibitor (aprotinin) of bovine origin. Two reconstituted components, sealer protein, and thrombin solutions are mixed and applied topically. The viscous solution quickly sets into an elastic coagulum.

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