Bleeding Disorder Treatments: Hemophilia B- MPR

Bleeding Disorder Treatments: Hemophilia B

BLEEDING DISORDER TREATMENTS: HEMOPHILIA B
Brand	Strength	Form	Dose
ANTI-INHIBITOR COAGULANT COMPLEX
Feiba^1,2	500 units,1000 units, 2500 units	lyophilized pwd for IV infusion after reconstitution	Adults and Children: Joint hemorrhage: 50–100units/kg every 12hrs until improved. Mucous membrane bleed: 50–100units/kg every 6hrs for ≥1 day or until resolved. Soft tissue hemorrhage: 100units/kg every 12hrs until resolved. Other severe hemorrhage (eg, CNS bleeds): 100units/kg every 6–12hrs until resolved. Pre-op: 50–100units/kg once immediately prior to surgery. Post-op: 50–100units/kg every 6–12hrs until resolved and healed. Routine prophylaxis: 85units/kg every other day. All: Max 200units/kg/day (100units/kg/dose).
COAGULATION FACTOR IX
AlphaNine SD⁷	500 IU, 1000 IU, 1500 IU	lyophilized concentrate for IV infusion after reconstitution	Adults: Dose (IU) = Body Weight (kg) × Desired Factor IX increase × 1 IU/kg. Individualize. Mild hemorrhage: increase FIX 20–30% (20–30 IU/kg twice daily) for 1–2 days or until resolved. Moderate hemorrhage: increase FIX 25–50% (25–50 IU/kg twice daily) for 2–7 days. Major hemorrhage: increase FIX 50% (30–50 IU/kg twice daily) for 3–5 days then maintain at 20% (20 IU/kg twice daily) for up to 10 days. Surgery: pre-op: increase FIX 50–100% (50–100 IU/kg twice daily), then maintain at 50–100% for 7–10 days or until healed. Max infusion rate: 10mL/min. Children: See full labeling.
BeneFix³	250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU	lyophilized pwd for IV infusion after reconstitution	Adults and Children: Initial Dose Required (IU) = Body Weight (kg) x Desired FIX increase x Reciprocal of observed recovery (IU/kg per IU/dL). Average recovery (≥12yrs): Dose Required (IU) = Body Weight (kg) x Desired FIX increase x 1.3 (IU/kg per IU/dL); (<12yrs): Dose Required (IU) = Body Weight (kg) x Desired FIX increase x 1.4 (IU/kg per IU/dL). Hemorrhage (Minor): 20–30% increase every 12–24hrs for 1–2 days; (Moderate): 25–50% increase every 12–24hrs for 2–7 days until resolved; (Major): 50–100% increase every 12–24hrs for 7–10 days. Routine prophylaxis: ≥16yrs: 100 IU/kg once weekly; adjust dose or frequency based on clinical response.
Ixinity	250 IU, 500 IU, 1000 IU, 1500 IU, 2000 IU, 3000 IU	lyophilized pwd for IV infusion after reconstitution	Adults and Children: <12yrs: Not established. Dosage Required (IU) = Body Weight (kg) x Desired Factor IX increase (% of normal or IU/dL) x reciprocal of observed recovery (IU/kg per IU/dL). ≥12yrs: Minor bleed: 30–60% increase every 24hrs for 1–3 days until healing achieved. Moderate: 40–60% increase every 24hrs for 2–7 days until healing achieved. Major: 60–100% increase every 12–24hrs for 2–14 days until healing achieved. Peri-op: Minor surgery (pre-op): 50–80% increase; (post-op): 30–80% increase every 24hrs for 1–5 days. Major (pre-op): 60–80% increase; (post-op): 20–40% increase every 8–24hrs for 7–14 days, or 30–50% increase every 8–24hrs for 4–6 days, or 40–60% increase every 8–24hrs for 1–3 days. Max infusion rate: 10mL/min.
Rebinyn⁴	500 IU, 1000 IU, 2000 IU	lyophilized pwd for IV infusion after reconstitution	Adults and Children: Minor/moderate bleed: 40 IU/kg as single dose. Major bleed: 80 IU/kg as single dose. Both: may give additional doses of 40 IU/kg. Peri-op: Minor: 40 IU/kg as single dose; repeat if needed; Major: 80 IU/kg as single dose; if needed, repeat doses of 40 IU/kg (in 1–3 day intervals) within first week, then may extend to once weekly until bleeding stops and healing achieved. Routine prophylaxis: 40 IU/kg once weekly.
Rixubis	250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU	lyophilized pwd for IV infusion after reconstitution	Adults: Initial dose = body weight (kg) x desired FIX increase × reciprocal of observed recovery (IU/dL per IU/kg). Dose in previously treated patients (IU) = body weight (kg) x desired FIX increase × 1.1 dL/kg. Individualize. Minor bleed: 20–30% required every 12–24hrs for ≥1 day until healing achieved. Moderate bleed: 25–50% required every 12–24hrs for 2–7 days until bleeding stops and healing achieved. Major bleed: 50–100% required every 12–24hrs for 7–10 days until bleeding stops and healing achieved. Peri-op: Minor: 30–60% required every 24hrs for ≥1 day until healing achieved; Major: 80–100% required every 8–24hrs for 7–10 days until bleeding stops and healing achieved. Routine prophylaxis (PTPs): 40–60 IU/kg twice weekly; titrate if needed. Max infusion rate 10mL/min. Children: Initial dose = body weight (kg) x desired FIX increase × reciprocal of observed recovery (IU/dL per IU/kg). Dose in PTPs (IU) = body weight (kg) x desired FIX increase x 1.4 dL/kg. Individualize. Minor, moderate, major bleed: see Adults: Peri-op: see Adults: Routine prophylaxis (PTPs): 60–80 IU/kg twice weekly; titrate if needed. Max infusion rate 10mL/min.
COAGULATION FACTOR IX/ALBUMIN FUSION PROTEIN
Idelvion⁵	250 IU, 500 IU, 1000 IU, 2000 IU, 3500 IU	lyophilized pwd for IV inj after reconstitution	Adults and Children: Dose (IU) = body weight (kg) × desired FIX increase x reciprocal of recovery (IU/dL per IU/kg). Individualize. Minor/moderate bleed: 30–60% required; repeat every 48–72hrs for ≥1 day until bleeding stops and healing achieved. Major bleed: 60–100% required; repeat every 48–72hrs for 7–14 days until bleeding stops and healing achieved; give maintenance dose weekly. Peri-op: Minor: 50–80% required; repeat every 48–72hrs for ≥1 day or until healing achieved; Major: initially 60–100% required every 48–72hrs for 7–14 days or until bleeding stops and healing achieved; may repeat every 48–72hrs for the 1st week or until healing achieved; give maintenance dose 1–2 times weekly. Routine prophylaxis (<12yrs): 40–55 IU/kg every 7 days; (≥12yrs): 25–40 IU/kg every 7 days (may give 50–75 IU/kg every 14 days if well tolerated). Max infusion rate: 10mL/min.
COAGULATION FACTOR IX/Fc FUSION PROTEIN
Alprolix⁶	250 IU, 500 IU, 1000 IU, 2000 IU, 3000 IU, 4000 IU	lyophilized pwd for IV inj after reconstitution	Adults and Children: Dose (IU) = body weight (kg) × desired FIX increase x reciprocal of recovery (IU/kg per IU/dL). Individualize. Minor/moderate bleed: 30–60% required; repeat every 48hrs if needed. Major bleed: 80–100% required; consider repeat dose after 6–10hrs, then every 24hrs for the 1^st 3 days; then may reduce dose and frequency to every 48hrs or longer until bleeding stops and healing achieved. Peri-op: Minor: 50–80% required as single dose; repeat as needed after 24–48hrs until bleeding stops and healing achieved; Major: initially 60–100% required; consider repeat dose after 6–10hrs, then every 24hrs for the 1^st 3 days; then may reduce dose and frequency to every 48hrs or longer until bleeding stops and healing achieved. Routine prophylaxis (<12yrs): initially 60 IU/kg once weekly; (≥12yrs): initially 50 IU/kg once weekly or 100 IU/kg once every 10 days. Max infusion rate 10mL/min.
COAGULATION FACTOR IX COMPLEX
Profilnine⁷	500 IU, 1000 IU, 1500 IU	lyophilized concentrate for IV infusion after reconstitution	Adults: Dosage Required (IU) = Body Weight (kg) × Desired Factor IX increase × 1 IU/kg. Minor to moderate hemorrhage: 20–30% FIX increase every 16–24hrs for 1–2 days (minor) or 2–7 days (moderate) until healing achieved. Major hemorrhage: 30–50% FIX increase every 16–24hrs for 3–10 days; then maintain FIX levels at 20% until healing achieved. Surgery (pre-op): 30–50% FIX increase every 16–24hrs for 7–10 days (increase FIX to 50% immediately prior to dental extractions); maintain FIX levels at 30–50% until healing achieved. Max infusion rate: 10mL/min. Children: Not established.
COAGULATION FACTOR VIIa
NovoSeven RT¹	1mg, 2mg, 5mg, 8mg	lyophilized pwd for IV inj after recon-stitution	Adults and Children: Give by IV bolus inj over 2–5mins. Bleeding: 90mcg/kg every 2hrs, adjust until hemostasis achieved; post-hemostatic dose: continue at 3–6hrs intervals for severe bleeds. Peri-op: initially 90mcg/kg prior to surgery, repeat at 2hr intervals during surgery. Post-op: Minor: every 2hrs for 48hrs, then every 2–6hrs until healed; Major: every 2hrs for 5 days, then every 4hrs or by continuous infusion at 50mcg/kg/hr, until healed; additional doses can be given.
Sevenfact¹	1mg, 5mg	lyophilized pwd for IV infusion after reconstitution	Adults: Give by IV bolus over ≤2mins. Mild/moderate bleeding: 75mcg/kg every 3hrs until hemostasis is achieved; or initially 225mcg/kg, if hemostasis is not achieved within 9hrs, additional 75mcg/kg may be given every 3hrs as needed to achieve hemostasis. Consider alternative treatments if bleeding is not controlled within 24hrs of first administration. Severe bleeding: initially 225mcg/kg, if hemostasis is not achieved after 6hrs, give 75mcg/kg every 2hrs until achieved. Both: cumulative daily doses >900mcg/kg have not been studied. Children: <12yrs: not established.
ETRANACOGENE DEZAPARVOVEC-DRLB
Hemgenix	1×10¹³ gc/mL	susp for IV infusion after dilution	Adults: 2×10¹³ gc/kg (2mL/kg). Infuse at rate 500mL/hr (8mL/min). Children: Not established.
NOTES
Key: FIX = Factor IX; gc = genome copies; PTP = previously treated patients ¹ For Hemophilia B with inhibitors. ² Contains Factors II, IX, X (non-activated); Factor VII (activated); Factor VIII inhibitor bypassing activity; Prothrombin Complex Factors. ³ May need higher doses if FIX inhibitors present or low FIX recovery. ⁴ GlycoPEGylated. ⁵ Dose adjustment may be needed in children <12yrs. ⁶ More frequent or higher doses may be needed in children <12yrs (esp. <6yrs). ⁷ Contains Factor II, Factor VII, Factor X (non-therapeutic levels in AlphaNine). Not an inclusive list of medications and/or official indications. Please see drug monograph at www.eMPR.com and/or contact company for full drug labeling. (Rev. 7/2023)

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