Clotting Factor Concentrates

  • General Information

    • Clotting factor concentrates are stored in blood bank between 2 & 80C and should not be stored in ward fridges.
    • blood bank release order is required to pick up clotting factors from blood bank
    • Occasionally there are slightly more/less units in the vial e.g. a 500 unit vial may have 513 units or 489 units. Treat the vial as 500 units and give total content. Do not discard any product.
    • Record batch numbers on the medication/intravenous order chart - most clotting factor concentrates will have a peel off sticker for this purpose.

    Factor VIII (8)

    Dosage

    • Calculated on the basis that 1 unit/kg of factor VIII leads to a rise in plasma factor VIII of approximately 2%.
    • EXAMPLE: 50 units/kg will increase factor VIII level by ~100%.
    • Doses are rounded UP to the nearest vial size.
    • The half life of factor VIII is 8-12 hours.
    • Refer also to RCH Clinical Practice Guideline Haemophilia

    Administration

    Factor VIII is generally administered as a slow bolus intravenous injection.

    Continuous infusion of Factor VIII is indicated for patients requiring admission for severe bleeds or surgical procedures.  Factor VIII replacement for such patients should be managed in consultation with the Haemophilia Unit or the Haematologist on call.  A continuous infusion of 3 units/kg/hour via syringe pump is commenced after a bolus loading dose. 


    EXAMPLE: 18 kg child on Recombinant Factor VIII/Advate:

    • Loading dose 50 units per kg = 1000 units (round UP to nearest vial size)
    • Continuous infusion
      • ~ 50 units per kg made up to total volume of 50ml
      • 750 units Advate in 20mls + 30 mls Normal Saline
      • 750 units in 50ml = 15 units/ml
      • 3 units/kg/hour = 54 units per hour = 3.6mls/hr

    As Factor VIII does not contain any antimicrobial agent, syringes for continuous infusion should be prepared so that the contents of the syringe will be infused in less than 24 hours (as in example above.)

    If a plasma Factor VIII level is clinically indicated, the sample should not be taken from the same intravenous line as the continuous infusion. Notify the laboratory to expect the sample.

    Recombinant Factor VIII

    • Genetically engineered Factor VIII.
    • The product of choice for the prevention and treatment of bleeding associated with Haemophilia A (Factor VIII deficiency).
    • Does NOT contain von Willebrand Factor and is not indicated for the treatment of bleeding in von Willebrand's disease.

    There are two Recombinant Factor VIII products available in Australia, see table below.
    Whilst it is recommended that patients maintain treatment with their established brand of FVIII product, in an emergency situation administration of any brand of recombinant FVIII is acceptable. 

    Recombinant FVIII
    Brand Name

    Vial Sizes (units)

    Diluent  Volume

    Reconstitution Guide

    XynthaTM

    250
    500
    1000
    2000

    4ml 0.9% NaCl

    Xyntha Dual Chamber Reconstitution Guide (pdf)

    KogenateFSTM

    Note: Kogenate will be phased out by mid 2015

    250
    500
    1000
    2000

    2.5ml WFI
    (250, 500, 1000 unit vials)

    5ml WFI
    (2000 unit vial)

    Using Kogenate® FS with BIO-SET® (pdf)         

    AdvateTM

    250
    500
    1000
    1500
    2000

    5ml WFI BaxjectII (pdf)

     

    Plasma derived Factor VIII and von Willebrand Factor (BiostateTM)

    • Derived from donated human plasma and contains Factor VIII (FVIII) and von Willebrand Factor (vWF).
    • Used to treat bleeding episodes and prevent surgical bleeding in patients with von Willebrands disease. Refer also to RCH Clinical Practice Guideline von Willebrand's Disease.
    • Rarely used for patients with Haemophilia A (recombinant FVIII is the product of choice). 
    • May be used in Haemophilia A patients with FVIII inhibitors
    •  Please note that the Biostate vial will state both Factor VIII and von Willebrand Factor units and the ratio of FVIII to vWF is 1:2
      • Make sure when ordering Biostate the dosage is ordered as FVIII units (or both FVIII and vWF units)
      • Eg 'Biostate 1000 FVIII units' or 'Biostate 1000 FVIII units (=2000 vWF units)'
    BiostateTM Vial Sizes
    (FVIII units/vWF units)

    Diluent Volume (Water For Injection)

    Reconstitution Guide

    250 FVIII/500 vWF
    500FVIII/1000 vWF
    1000FVIII/2000 vWF

    5ml
    10ml
    10ml

    Click here (pdf)

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    Factor IX (9)

    Dosage

    • Calculated on the basis that 1 unit/kg of factor IX leads to a rise in plasma factor IX of approximately 0.7 - 1%.
    • EXAMPLE 50 units/kg will increase factor IX level by ~35-50%
    • Doses are rounded UP to the nearest vial size.
    • The half life of factor IX is 18-24 hours.
    • Refer also to RCH Clinical Practice Guideline Haemophilia.

    Administration

    Factor IX is generally administered as a slow bolus intravenous injection.

    Continuous infusion of Factor IX is indicated for patients requiring admission for severe bleeds or surgical procedures.  Factor IX replacement for such patients should be managed in consultation with the Haemophilia Unit or the Haematologist on call.  A continuous infusion of 3 units/kg/hour via syringe pump is commenced after a bolus loading dose.


    EXAMPLE: 18 kg child on Recombinant Factor IX / Benefix:

    • Loading dose 100 units per kg = 2000 units (round UP to nearest vial size)
    • Continuous infusion
      • ~ 50 units per kg made up to total volume of 50ml
      • 750 units Benefix in 10mls + 40 mls Normal Saline
      • 750 units in 50ml = 15 units/ml
      • 3 units/kg/hour = 54 units per hour = 3.6 mls/hr

    As Factor IX does not contain any antimicrobial agent, syringes for continuous infusion should be prepared so that the contents of the syringe will be infused in less than 24 hours (as in example above.)

    If a plasma Factor IX level is clinically indicated, the sample should not be taken from the same intravenous line as the continuous infusion.  Notify the laboratory to expect the sample.

    Precaution: Haemophilia B patients with inhibitors (antibodies to Factor IX) are at risk of severe allergic reactions, including anaphylaxis, to Factor IX.  Haemophilia patients are monitored for the development of inhibitors and inhibitors to Factor IX are rare.

    Recombinant Factor IX (BenefixTM)

    • Genetically engineered Factor IX.
    • The product of choice for the prevention and treatment of bleeding associated with Haemophilia B (Factor IX deficiency).
    BenefixTM Vial Sizes (units)

    Diluent Volume (0.234%NaCl) 

    Reconstitution Guide

    250
    500
    1000
    2000

    5ml
    5ml
    5ml
    5ml

     Click here (pdf)

    Plasma derived Factor IX (MonofixTM)

    • Derived from donated human plasma and contains Factor IX.
    • Rarely used for the treatment of Haemophilia B (recombinant Factor IX is the product of choice).
    • May be used in Haemophilia B patients with FIX inhibitors.

    MonofixTM Vial Sizes (units)

    Diluent Volume (Water For Injection) 

    Reconstitution Guide

    500
    1000

    10ml
    10ml

    Mix2Vial - 5 easy steps (pdf)

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    Activated Factor VII (7) Novoseven®RT

    • Recombinant activated factor VII (7).
    • Indicated and licensed for the treatment of bleeding episodes in haemophilia patients with inhibitors (antibodies) to factors VIII or IX.
    • Has been used 'off label' in the management of intractable bleeding post trauma or surgery, which has not been controlled by surgery or conventional transfusion replacement therapy
    • Clinical Haematology Department/Haematologist on call must be consulted prior to use of this product

    Dosage

    • According to individual treatment plan for haemophilia patients with Factor VIII or IX inhibitors (usually multiple doses of 90 microgram/kg).
    • 90 micrograms/kg for intractable bleeding not responsive to surgical or transfusion replacement therapy (larger doses, up to 180 micrograms/kg may be appropriate in neonatal patients)
    • 10 micrograms/kg for treatment of bleeding episodes in patients with factor VII deficiency (rare).
    • The half life of activated Factor VII is 2 hours.
    • After reconstitution with the supplied diluent each vial contains 1.0mg/ml Factor VIIa.

    Administration

    • Novoseven®RTis stable for 24 hours after reconstitution but should be administered as soon as possible to avoid microbial contamination
    • If not used immediately after reconstitution, Novoseven®RT should be stored in the vial (not in the syringe) in a refrigerator at 20C-80C for no longer than 24 hours
    • Administer as a bolus intravenous injection over 2-5 minutes.
    • Do not mix with other intravenous solutions or medications.
    • Administer as close to the IV cannula as possible.
    • Flush with Normal Saline pre and post administration.

    Novoseven®RT Vial Sizes

    Diluent (10 mmol L-histidine in Water For Injection) Volume

    Concentration
    of
    Reconstituted Product

    Reconstitution Guide

    1 mg
    2 mg
    5 mg

    1.1 ml
    2.1 ml
    5.2 ml

    1mg/ml
    1mg/ml
    1mg/ml

    See product insert

     

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    Factor XIII (13) FibrogamminTM

    • Plasma derived factor XIII.
    • Used primarily for prevention and treatment of bleeding in Factor XIII deficiency (rare).
    • Administered as a slow bolus intravenous injection.
    • The half life of factor XIII is 8 to 10 days.

    FibrogamminTM Vial Sizes (units)

    Diluent Volume (Water For Injection)

    Reconstitution Guide

    250
    1250

    4ml
    20ml

    See product insert.

     

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    ProthrombinexTM

    • A plasma derived prothrombin complex concentrate which contains factors II, IX and X.
    • NOT used in patients with Haemophilia B (Factor IX deficiency) as purified Factor IX concentrates are superior.
    • Used for the prevention and treatment of bleeding in patients with low levels of factor II or factor X (rare) and in some patients who require reversal of anticoagulant therapy.
    • The use of Prothrombinex with antifibrinolytics (tranexamic acid) is not recommended due to the theoretical increase in risk of thrombosis.
    • Administered as a slow bolus IV injection.

    ProthrombinexTM Vial Sizes (units)

    Diluent Volume (Water For Injection)

    Reconstitution Guide

    500

    20ml

    Click here (pdf)

     

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    Factor XI (Eleven) BPLTM

    • Plasma derived factor XI (Eleven)
    • The half life of factor XI is approximately 2 days.
    • Use of factor XI concentrates, particularly in high doses, has been associated with thrombosis
    • Dosage should be determined in consultation with Clinical Haematology
    • Administered as a slow bolus intravenous injection.
    • Used primarily for prevention and treatment of bleeding in Factor XI deficiency (rare)

    Dried Factor XI (Eleven) Fraction BPLTM Vial Sizes (units)

    Diluent Volume (Water For Injection)

    Reconstitution Guide

    1000

    (note that actual factor XI content is also stated on each vial and may vary between batches)

    10ml

    See product insert.

     

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    Fibrinogen Concentrate (RiaSTAP®)

    • Plasma derived fibrinogen (Factor I) concentrate
    • The half life of fibrinogen is approximately 2 - 4 days
    •  Available for the prevention and treatment of bleeding in patients with congenital fibrinogen deficiency only (this condition is  rare)
    •  Use of RiaSTAP® and dosage should be determined in consultation with Clinical Haematology
    •  Administered by slow intravenous injection at a rate not exceeding 5 ml per minute

     

    RiaSTAP® Vial Sizes

    Diluent Volume (Water For Injection)

    Reconstitution Guide

    1 gram

    50ml (not supplied with product)

    See product insert.

      

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