No

Antihemophilic Agent; Blood Product Derivative

Control bleeding in patients with factor IX deficiency (hemophilia B or Christmas disease) NOTE: Factor IX concentrate containing ONLY factor IX is also available and preferable for this indication.

Prevention/control of bleeding in hemophilia A patients with inhibitors to factor VIII

Prevention/control of bleeding in patients with factor VII deficiency

Emergency correction of the coagulopathy of warfarin excess in critical situations.

C

Liver disease with signs of intravascular coagulation or fibrinolysis, not for use in factor VII deficiencies, patients undergoing elective surgery

Use with caution in patients with liver dysfunction; prepared from pooled human plasma - the risk of viral transmission is not totally eradicated; monitor patients who receive repeated doses twice daily with PTT and prothrombin time and level of factor being replaced (eg, usually VII or IX); if PT is <10 seconds, this may indicate risk of hypercoagulable complication

1% to 10%:

Central nervous system: Fever, headache, chills

Neuromuscular & skeletal: Tingling

Miscellaneous: Following rapid administration: Transient fever

<1%: Flushing, DIC, thrombosis following high dosages because of presence of activated clotting factors, tightness in chest, somnolence, urticaria, nausea, vomiting, tightness in neck

Symptoms of overdose include disseminated intravascular coagulation (DIC)

Increased toxicity: Do not coadminister with aminocaproic acid may increase risk for thrombosis

When stored at refrigerator temperature, 2°C to 8°C (36°F to 46°F), Coagulation Factor IX is stable for the period indicated by the expiration date on its label. Avoid freezing which may damage container for the diluent.

Standard diluent: Dose in units/bag

Minimum volume: Use complete vial(s) for entire dose

Comments: Infusion rate should be 2 mL/minute

Replaces deficient clotting factor including factor X; hemophilia B, or Christmas disease, is an X-linked recessively inherited disorder of blood coagulation characterized by insufficient or abnormal synthesis of the clotting protein factor IX. Factor IX is a vitamin K-dependent coagulation factor which is synthesized in the liver. Factor IX is activated by factor XIa in the intrinsic coagulation pathway. Activated factor IX (IXa), in combination with factor VII:C activates factor X to Xa, resulting ultimately in the conversion of prothrombin to thrombin and the formation of a fibrin clot. The infusion of exogenous factor IX to replace the deficiency present in hemophilia B temporarily restores hemostasis.

Half-life:

IX component: 24 hours

Children and Adults: Dosage is expressed in units of factor IX activity and must be individualized. I.V. only:

Total blood volume (mL blood/kg) = 70 mL/kg (adults), 80 mL/kg (children)

Plasma volume = total blood volume (mL) x [1 - Hct (in decimals)]

For example, for a 70 kg adult with a Hct = 40%: Plasma volume = [70 kg x 70 mL/kg] x [1 - 0.4] = 2940 mL

To calculate number of units needed to increase level to desired range (highly individualized and dependent on patient's condition): Number of units = desired level increase [desired level - actual level] x plasma volume (in mL)

For example, for a 100% level in the above patient who has an actual level of 20%: Number of units needed = [1 (for a 100% level) - 0.2] x 2940 mL = 2352 units

As a general rule, the level of factor IX required for treatment of different conditions is listed below:

Minor Spontaneous Hemorrhage, Prophylaxis:

Desired levels of factor IX for hemostasis: 15% to 25%

Initial loading dose to achieve desired level: <20-30 units/kg

Frequency of dosing: Once; repeated in 24 hours if necessary

Duration of treatment: Once; repeated if necessary

Major Trauma or Surgery:

Desired levels of factor IX for hemostasis: 25% to 50%

Initial loading dose to achieve desired level: <75 units/kg

Frequency of dosing: q18-30h, depending on half-life and measured factor IX levels

Duration of treatment: Up to 10 days, depending upon nature of insult

Factor VIII inhibitor patients: 75 units/kg/dose; may be given every 6-12 hours

Anticoagulant overdosage: I.V.: 15 units/kg

I.V. administration only; rate of administration should not exceed 10 mL/minute; use filter needle to draw product into syringe

Levels of factors being replaced (eg, VII or IX), PT, PTT

Average normal factor VII and factor IX levels are 50% to 150%; patients with severe hemophilia will have levels <1%, often undetectable. Moderate forms of the disease have levels of 1% to 10% while some mild cases may have 11% to 49% of normal factor IX.

In preparation for and following surgery:

Level to prevent spontaneous hemorrhage: 5%

Minimum level for hemostasis following trauma and surgery: 30% to 50%

Severe hemorrhage: >60%

Major surgery: >60% prior to procedure, 30% to 50% for several days after surgery, and >20% for 7-10 days thereafter

No information available to require special precautions

No effects or complications reported

Early signs of hypersensitivity reactions including hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and anaphylaxis indicate discontinuation of use of the concentrate and physician should be contacted if these symptoms occur

Injection:

BeneFix™: 250 units, 500 units, 1000 units

Konyne® 80: 20 mL, 40 mL

Hemonyne®: 20 mL, 40 mL

Profilnine® SD: Single dose vial

Proplex® T: 30 mL vial

Lusher JM, "Thrombogenicity Associated With Factor IX Complex Concentrates," Semin Hematol, 1991, 28(3 Suppl 6):3-5.