Trade Names:AlphaNine SD- Injection, lyophilized powder for solution of dried plasma fraction of factor IX (human)
Trade Names:Bebulin VH- Injection, lyophilized powder for solution of purified freeze-dried concentrate of coagulation factors IX, II, X, and low amounts of VII (human), heat-treated
Trade Names:BeneFIX- Injection, lyophilized powder for solution of factor IX, recombinant
Trade Names:Mononine- Powder Injection, lyophilized powder for solution of factor IX (human), plasma-derived
Trade Names:Profilnine SD- Injection, lyophilized powder for solution of factors IX, II, X, and low amount of VII (human), plasma-derived
Immunine VH (Canada)Restores hemostasis in patients with factor IX (FIX) deficiency.
Mean biologic half-life is 18.8 to 25.3 h (adults) and approximately 20.2 h (children).
Control and prevention of hemorrhagic episodes in patients with FIX deficiency (hemophilia B, Christmas disease).
BenefixPerioperative management in adults and children with hemophilia B.
None known.
BeneFIXHypersensitivity to hamster protein.
MononineHypersensitivity to mouse protein.
IV
AlphaNine SDThe method of calculating the AlphaNine SD FIX dose is as follows: Number of FIX units required equals body weight (kg) × desired increase in plasma FIX (percent of normal) × 1 unit/kg. The following are treatment guidelines using AlphaNine SD for hemorrhagic events and surgery in patients diagnosed with hemophilia B:
Minor hemorrhage (eg, bruises, cuts or scrapes, uncomplicated joint hemorrhage)FIX levels should be brought to at least 20% to 30% (20 to 30 units of FIX/kg twice daily) until hemorrhage stops and healing has been achieved (1 to 2 days).
Moderate hemorrhages (eg, nose bleeds, mouth and gum bleeds, dental extractions, hematuria)FIX levels should be brought to 25% to 50% (25 to 50 units of FIX/kg twice daily) until healing has been achieved (2 to 7 days, or average).
Major hemorrhage (eg, joint and muscle hemorrhage [especially in the large muscles], major trauma, hematuria, intracranial and intraperitoneal bleeding)FIX levels should be brought to 50% for at least 3 to 5 days (30 to 50 units of FIX/kg twice daily). Following this treatment period, FIX levels should be maintained at 20% (20 units of FIX/kg twice daily) until healing has been achieved. Major hemorrhages may require treatment for up to 10 days.
SurgeryPrior to surgery, FIX should be brought to 50% to 100% of normal (50 to 100 units of FIX/kg twice daily). For the next 7 to 10 days, or until healing has been achieved, the patient should be maintained at 50% to 100% FIX levels (50 to 100 units of FIX/kg twice daily).
Bebulin VHThe method of calculating the Bebulin VH FIX dose is as follows: Number of FIX units required equals body weight (kg) × desired FIX increase (percent of normal) × 1.2. The following are treatment guidelines using Bebulin VH for hemorrhagic events and surgery in patients diagnosed with hemophilia B:
Minor hemorrhage (eg, early hemarthrosis, minor epistaxis and gingival bleeding, mild hematuria)To achieve an approximate FIX level of 20% of normal, the typical initial dose is 25 to 35 units/kg for 1 day.
Moderate hemorrhage (eg, severe joint bleeding; early hematoma; major open bleeding; minor trauma; minor hemoptysis, hematemesis, and melena; major hematuria)To achieve an approximate FIX level of 40% of normal, the typical initial dose is 40 to 55 units/kg for 2 days or until adequate wound healing.
Major hemorrhage (eg, severe hematoma; major trauma; severe hemoptysis, hematemesis, and melena)To achieve an approximate FIX level of at least 60% of normal, the typical initial dose is 60 to 70 units/kg for 2 to 3 days or until adequate wound healing.
Major surgeryOn the day of surgery, to achieve an approximate FIX level of at least 60% of normal, administer 70 to 95 units/kg. During the initial postoperative period (first and second week), to achieve an approximate FIX level of 60% to 20% of normal, administer 70 to 35 units/kg. During the late postoperative period (from third week onwards), to achieve an approximate FIX level of 20% of normal, administer 35 to 25 units/kg.
Minor surgeryOn the day of surgery, to achieve an approximate FIX level of 40% to 60% of normal, administer 50 to 60 units/kg. During the initial postoperative period (first and second week), to achieve an approximate FIX level of 40% to 20% of normal, administer 55 to 25 units/kg. Dosing guidelines for the late postoperative period (from third week onwards) are not applicable.
Long-term prophylaxis20 to 30 units/kg administered once, or preferably up to twice weekly, have been shown to reduce frequency of spontaneous hemorrhage. However, prophylactic dosage regimens should be tailored to individual needs.
BeneFIXThe method of calculating the BeneFIX dose is as follows:
The following are treatment guidelines using BeneFIX for hemorrhagic events and surgery in patients diagnosed with hemophilia B:
Minor hemorrhage (eg, uncomplicated hemarthrosis, superficial muscle, soft tissue)For the required circulating FIX activity of 20% to 30% (or units/dL), the dosing interval should be 12 to 24 h for a duration of 1 to 2 days.
Moderate hemorrhage (eg, intramuscle or soft tissue with dissection, mucous membranes, dental extractions, hematuria)For the required circulating FIX activity of 25% to 50% (or units/dL), the dosing interval should be 12 to 24 h administered until bleeding stops and healing begins (about 2 to 7 days).
Major hemorrhage (eg, pharynx, retropharynx, retroperitoneum, CNS, surgery)For the required circulating FIX activity of 50% to 100% (or units/dL), the dosing interval should be 12 to 24 h for a duration of 7 to 10 days.
MononineThe method of calculating the Mononine FIX dose is as follows: Number of FIX units required equals body weight (kg) × desired FIX increase (percent or units/dL) × 1 unit/kg (or units/dL). The following are treatment guidelines using Mononine for hemorrhagic events and surgery in patients diagnosed with hemophilia B:
Minor spontaneous hemorrhage, prophylaxisFor desired levels of FIX for hemostasis of 15% to 25% (or units/dL), administer an initial loading dose of up to 20 to 30 units/kg, which may be repeated once in 24 h if needed. The duration of treatment is once and may be repeated if needed.
Major trauma or surgeryFor desired levels of FIX for hemostasis of 25% to 50% (or units/dL), administer an initial loading dose of up to 75 units/kg, which may be repeated every 18 to 30 h depending on half-life and measured FIX levels. The duration of treatment is up to 10 days, depending upon nature of insult.
Profilnine SDThe method of calculating the Profilnine SD FIX dose is as follows: Number of FIX units required equals body weight (kg) × desired increase in plasma FIX (percent of normal) × 1 unit/kg. The following are treatment guidelines using Profilnine SD for hemorrhagic events and surgery in patients diagnosed with hemophilia B.
Mild to moderate hemorrhageMay usually be treated with a single administration sufficient to raise plasma FIX level to 20% to 30%.
Severe hemorrhageRaise plasma FIX to 30% to 50%. Infusions are generally required daily.
SurgeryFIX level should be raised to 30% to 50% for at least 1 week following surgery.
Dental extractionsFIX level should be raised to 50% immediately prior to the procedure. Administer additional FIX complex if bleeding occurs.
BeneFIX may be stored at room temperature (not exceeding 77°F) for up to 6 mo. Profilnine SD may be stored at room temperature (not exceeding 86°F) for up to 3 mo. Mononine may be stored at room temperature (not exceeding 77°F) for up to 1 mo. AlphaNine SD may be stored at room temperature (not exceeding 86°F) for up to 1 mo. Store Bebulin VH at 35° to 46°F; avoid freezing.
May increase risk of thrombosis.
None well documented.
Deep vein thrombosis, hypotension, superior vena cava syndrome, thrombophlebitis, thrombosis (postmarketing).
Headache (11%); dizziness (8%); somnolence (3%); drowsiness, shaking (2%); lethargy.
Rash (8%); hives (5%); urticaria.
Allergic rhinitis (5%); blurred vision, visual disturbances (2%).
Nausea (6%); taste perversion (5%); diarrhea, vomiting (2%).
Renal infarct (2%).
Anaphylaxis, angioedema, laryngeal edema (postmarketing).
Elevated ALT or AST, increased alkaline phosphatase (2%).
Injection-site pain (6%); cellulitis or phlebitis at IV site, injection-site reaction (2%).
Dyspnea (3%); dry cough, lung disorder (2%).
FIX inhibitor, fever, flushing, shaking (3%); allergic reaction, asthma, burning sensation in jaw and skull, chest tightness, chills, hepatitis A virus seroconversion, hypoxia, parvovirus B19 seroconversion, photosensitivity (2%); tingling; cyanosis, inadequate FIX recovery, inadequate therapeutic response, inhibitor development (postmarketing).
Category C .
Undetermined.
Safety and efficacy of Profilnine SD and AlphaNine SD have not been established in children 16 yr of age and younger. A Mononine small trial showed safety and effectiveness in patients from 1 day to 20 years of age with excellent hemostasis without thrombotic complications.
Studies did not include sufficient numbers of patients 65 yr of age and older to determine if they respond differently from younger patients. Dose selection should be individualized.
Allergic-type hypersensitivity reactions, including anaphylaxis, have been reported. Evaluate patient experiencing allergic reaction for presence of FIX inhibitor.
Nephrotic syndrome has been reported following immune tolerance induction with FIX concentrates in hemophilia B patients with FIX inhibitors and a history of allergic reactions to FIX. Safety and efficacy of using BeneFIX for immune tolerance induction have not been established.
Diluent vial for BeneFIX packaging may contain dry natural latex rubber that may cause allergic reactions in latex-sensitive individuals.
If expected plasma factor IV activity levels are not attained, or if bleeding is not controlled with the expected dose, an assay to measure FIX inhibitor concentration should be performed. Patients with FIX inhibitors may be at increased risk of anaphylaxis upon subsequent challenge with FIX.
Because use of FIX complex concentrates has been associated with development of thromboembolic complications, the use of FIX-containing products may be potentially hazardous in patients with signs of fibrinolysis or with DIC. Use with caution when administering to patients with liver disease, patients postoperatively, neonates, or to patients at risk of thromboembolic phenomena or DIC.
All products except BeneFIX are prepared from pooled units of human plasma and may carry a risk of transmitting infectious agents.
No symptoms have been reported.
Copyright © 2009 Wolters Kluwer Health.