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Emicizumab-kxwh

Medically reviewed on Sep 10, 2018

Pronunciation

(em i SIZ ue mab kxwh)

Index Terms

  • Hemlibra

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution, Subcutaneous [preservative free]:

Hemlibra: 30 mg/mL (1 mL); 60 mg/0.4 mL (0.4 mL); 105 mg/0.7 mL (0.7 mL); 150 mg/mL (1 mL)

Brand Names: U.S.

  • Hemlibra

Pharmacologic Category

  • Antihemophilic Agent
  • Monoclonal Antibody

Pharmacology

Emicizumab-kxwh, a humanized monoclonal modified immunoglobulin G4 (IgG4) antibody with a bispecific factor IXa- and factor X-directed antibody, bridges activated factor IX and factor X to restore the function of missing activated factor VIII that is needed for effective hemostasis. Emicizumab-kxwh has no structural relationship or sequence homology to FVIII and, therefore, does not induce or enhance the development of direct inhibitors to FVIII.

Distribution

Vd: 11.4 L

Half-Life Elimination

27.8 ± 8.1 days

Special Populations Note

Body weight: Clearance and volume of distribution increase with increasing body weight (14.2 kg to 131 kg). Dosing in mg/kg provides similar emicizumab-kxwh exposure across body weight range.

Use: Labeled Indications

Hemophilia A, prophylaxis: Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in patients with hemophilia A (congenital factor VIII deficiency) with factor VIII inhibitors

Contraindications

There are no contraindications listed in the manufacturer's labeling.

Dosing: Adult

Hemophilia A, prophylaxis: SubQ: Initial: 3 mg/kg once weekly for 4 weeks, then 1.5 mg/kg once weekly thereafter

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

Hemophilia A, prophylaxis: Infants, Children, and Adolescents: SubQ: Initial: Refer to adult dosing.

Dosing: Renal Impairment

There are no dosage adjustments provided in the manufacturer's labeling.

Dosing: Hepatic Impairment

There are no dosage adjustments provided in the manufacturer's labeling.

Administration

SubQ: For SubQ administration. Do not shake. Administer immediately after removal from vial. Rotate injection sites (upper outer arms, thighs, or any quadrant of abdomen). Do not inject into moles, scars, or areas where the skin is tender, bruised, red, hard, or not intact. Patient may self-inject, or the patient’s caregiver may administer; however, only a health care provider should administer in the upper outer arm. Self-administration is not recommended for children <7 years of age.

Refer to product information for preparation and additional administration techniques. Do not use different vials of different concentrations when combining vials to administer prescribed dose.

Storage

Store at 2°C to 8°C (36°F to 46°F); protect from light. Do not freeze. May also store unopened vials at <30°C (<86°F) for up to 7 days. Once removed from the vial, discard if not used immediately. Discard unused solution.

Drug Interactions

Anti-inhibitor Coagulant Complex (Human): Emicizumab-kxwh may enhance the thrombogenic effect of Anti-inhibitor Coagulant Complex (Human). Monitor therapy

Belimumab: Monoclonal Antibodies may enhance the adverse/toxic effect of Belimumab. Avoid combination

Factor VIIa (Recombinant): Emicizumab-kxwh may enhance the thrombogenic effect of Factor VIIa (Recombinant). Monitor therapy

Factor VIII Products: Emicizumab-kxwh may enhance the thrombogenic effect of Factor VIII Products. Monitor therapy

Test Interactions

Emicizumab-kxwh affects intrinsic pathway clotting-based laboratory tests, including activated clotting time (ACT), activated partial thromboplastin time (aPTT), and all assays based on aPTT, such as one-stage factor VIII (FVIII) activity. Do not use intrinsic pathway clotting based laboratory test results to monitor emicizumab-kxwh.

Adverse Reactions

>10%:

Central nervous system: Headache (15%)

Immunologic: Antibody development (≤22%)

Local: Injection site reaction (19%)

1% to 10%:

Dermatologic: Injection site pruritus (5%)

Gastrointestinal: Diarrhea (6%)

Local: Erythema at injection site (7%), pain at injection site (5%)

Neuromuscular & skeletal: Arthralgia (10%), myalgia (5%)

Miscellaneous: Fever (7%)

ALERT: U.S. Boxed Warning

Thrombotic microangiopathy and thromboembolism:

Cases of thrombotic microangiopathy and thrombotic events were reported when on average a cumulative amount of >100 units/kg/24 hours of activated prothrombin complex concentrate (aPCC) was administered for 24 hours or more to patients receiving emicizumab-kxwh prophylaxis. Monitor for the development of thrombotic microangiopathy and thrombotic events if aPCC is administered. Discontinue aPCC and suspend dosing of emicizumab-kxwh if symptoms occur.

Warnings/Precautions

Concerns related to adverse effects:

• Thrombotic microangiopathy and thromboembolism: [US Boxed Warning]: Cases of thrombotic microangiopathy and thrombotic events were reported when on average a cumulative amount of >100 units/kg/24 hours of activated prothrombin complex (aPPC) concentrate was administered for ≥24 hours to patients receiving emicizumab-kxwh prophylaxis. Monitor for the development of thrombotic microangiopathy and thrombotic events if aPCC is administered. Discontinue aPCC and suspend dosing of emicizumab-kxwh if symptoms occur. Patients presented with thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury, without severe deficiencies in ADAMTS13 activity; improvement of thrombotic microangiopathy and thromboembolism was seen within 1 week and 1 month, respectively, following discontinuation of aPCC. If these complications occur, discontinue aPCC immediately and interrupt emicizumab-kxwh; consider benefits vs risks of resuming emicizumab-kxwh following complete resolution of thrombotic microangiopathy and thrombotic events.

Concurrent drug therapy issues:

• Drug-drug interactions: Potentially significant interactions may exist, requiring dose or frequency adjustment, additional monitoring, and/or selection of alternative therapy. Consult drug interactions database for more detailed information.

Monitoring Parameters

Monitor for thrombotic microangiopathy and thrombotic events if aPCC is administered with emicizumab-kxwh.

Monitor coagulation parameters using single factor assays utilizing chromogenic or immuno-based methods. Chromogenic FVIII activity tests may be manufactured with either human or bovine coagulation proteins; human coagulation factors assays may overestimate the clinical hemostatic potential of emicizumab-kxwh; however, bovine coagulation factors assays are insensitive to emicizumab-kxwh (no activity measured) and can be used to monitor endogenous or infused FVIII activity, or to measure anti-FVIII inhibitors. Emicizumab-kxwh will produce a false negative result in clotting-based Bethesda assays for functional inhibition of FVIII; a chromogenic Bethesda assay utilizing a bovine-based FVIII chromogenic test that is insensitive to emicizumab-kxwh may be used. Due to the long half-life of emicizumab-kxwh, effects on coagulation assays may persist ≤6 months after the last dose.

Pregnancy Considerations

Animal reproduction studies have not been conducted. Emicizumab-kxwh is a humanized monoclonal immunoglobulin; endogenous IgG crosses the placenta.

Females of childbearing potential should use effective contraception during therapy.

Patient Education

• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)

• Patient may experience muscle pain, joint pain, or diarrhea. Have patient report immediately to prescriber signs of blood clots (numbness or weakness on one side of the body; pain, redness, tenderness, warmth, or swelling in the arms or legs; change in color of an arm or leg; angina; shortness of breath; tachycardia; or coughing up blood), confusion, passing out, weakness, back pain, abdominal pain, vision changes, angina, coughing up blood, eye pain, eye edema, tachycardia, facial numbness, extremity pain, shortness of breath, swelling of the arms or legs, difficult urination, nausea, vomiting, severe headache, jaundice, or severe injection site irritation (HCAHPS).

• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.

Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for health care professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience, and judgment in diagnosing, treating, and advising patients.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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