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Elapegademase

Medically reviewed by Drugs.com. Last updated on July 11, 2020.

Pronunciation

(EL a peg AD e mase)

Index Terms

  • Elapegademase-lvlr

Dosage Forms

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

Solution, Intramuscular [preservative free]:

Revcovi: elapegademase-lvlr 2.4 mg/1.5 mL (1.5 mL)

Brand Names: U.S.

  • Revcovi

Pharmacologic Category

  • Enzyme

Pharmacology

Adenosine deaminase is an enzyme that catalyzes the deamination of both adenosine and deoxyadenosine. Hereditary lack of adenosine deaminase activity results in severe immunodeficiency disease, an often fatal disorder. Elapegademase is an exogenous source of adenosine deaminase enzyme that reduces levels of toxic adenosine and deoxyadenosine and increases lymphocytes.

Time to Peak

27.2 to 72 hours

Use: Labeled Indications

Adenosine deaminase severe combined immune deficiency: Treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) in pediatric and adult patients.

Contraindications

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

Dosing: Adult

Adenosine deaminase severe combined immune deficiency: IM: Note: Optimal long-term dose and administration schedule should be individualized based on laboratory values (eg, adenosine deaminase [ADA] activity, trough deoxyadenosine nucleotides [dAXP] concentration) and clinical response.

Pegademase bovine naive patients:Initial: 0.2 mg/kg (based on ideal body weight or actual body weight, whichever is greater) twice weekly for a minimum of 12 to 24 weeks. After immune reconstitution is achieved, adjust dose to maintain trough ADA activity >30 mmol/hour/L, trough dAXP <0.02 mmol/L, and/or to maintain immune reconstitution based on clinical response.

Patients transitioning from pegademase bovine:

Pegademase bovine weekly dose unknown or dose ≤30 units/kg: Initial: Minimum of 0.2 mg/kg once weekly

Pegademase bovine weekly dose >30 units/kg: Initial: Calculate dose based on following formula:

Elapegademase dose (mg/kg) = (pegademase bovine dose [units/kg])/150

Increase dose by increments of 0.033 mg/kg once weekly if ADA trough activity <30 mmol/hour/L, trough dAXP >0.02 mmol/L, and/or immune reconstitution is inadequate based on clinical response; may divide total weekly dose into multiple administrations during a week

Dosing: Pediatric

Adenosine deaminase severe combined immune deficiency (ADA-SCID): Note: Treatment should be individualized and based on laboratory values (eg, adenosine deaminase [ADA] activity, deoxyadenosine nucleotides [dAXP] concentrations) and clinical response; the youngest patient treated in a study was 3 months of age.

Pegademase bovine (Adagen)-naive patients: Note: Dose should be based on actual body weight or ideal body weight (whichever is greater):

Infants, Children, and Adolescents: IM: 0.4 mg/kg/week divided into 2 weekly doses (0.2 mg/kg/dose twice weekly); continue for a minimum of 12 to 24 weeks until immune reconstitution is achieved. Following achievement of immune reconstitution, adjust dose down to maintain trough ADA activity >30 mmol/hour/L, trough dAXP concentration <0.02 mmol/L, and/or to maintain immune reconstitution based on clinical assessment.

Conversion from pegademase bovine (Adagen) to elapegademase (Revcovi):

Infants, Children, and Adolescents:

Initial dose:

Previous pegademase bovine (Adagen) dose unknown or ≤30 units/kg: IM: 0.2 mg/kg/week of elapegademase administered once weekly.

Previous pegademase bovine (Adagen) dose >30 units/kg: Calculate weekly elapegademase dose based on the following equation; may administer calculated dose once weekly or may divide into multiple doses in a week: IM:

Elapegademase dose (mg/kg/week) = Pegademase bovine (Adagen) dose (units/kg/week)/150

Subsequent elapegademase doses: IM: Increase dose by increments of 0.033 mg/kg/week if trough ADA activity <30 mmol/hour/L, trough dAXP level >0.02 mmol/L, and/or if immune reconstitution is inadequate based on clinical assessment.

Reconstitution

Prior to administration, remove from refrigerator and allow to warm to room temperature for 30 minutes. Solution is clear and colorless; do not use if cloudy, discolored or contains particulate matter. Solution should not be diluted or mixed with any other drugs. Draw solution from vial with ≥25 gauge needle; change the needle to patient appropriate size and gauge prior to administration. Discard unused medication.

Administration

IM: Administer intramuscularly; do not administer IV or SubQ. Do not dilute or mix with other medications prior to administration. Administer immediately after syringe preparation with an appropriate needle (size and gauge) for IM administration for the patient. Avoid administration near an artery or nerve. Periodically alternate injection site.

Storage

Refrigerate at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light; do not freeze or shake. Do not use if previously frozen.

Drug Interactions

Pegloticase: May diminish the therapeutic effect of PEGylated Drug Products. Monitor therapy

Pegvaliase: PEGylated Drug Products may enhance the adverse/toxic effect of Pegvaliase. Specifically, the risk of anaphylaxis or hypersensitivity reactions may be increased. Monitor therapy

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.

Frequency not defined:

Central nervous system: Abnormal gait, fatigue, migraine, seizures

Dermatologic: Facial swelling, fungal skin infection, skin rash

Gastrointestinal: Dental caries, diarrhea, gastrointestinal infection, hematochezia, nausea, oral candidiasis, tooth loss (extraction), upper abdominal pain, vomiting

Hematologic & oncologic: Lymphadenopathy, neutropenia, pulmonary hemorrhage

Infection: Abscess (groin), infection (stoma), influenza, tooth abscess

Local: Discomfort at injection site

Neuromuscular & skeletal: Arthralgia, asthenia

Ophthalmic: Conjunctivitis

Otic: Ear residue, debris, or precipitate; ear sign or symptom (canal irritation); otitis externa

Renal: Nephrolithiasis

Respiratory: Cough, ENT infection (ear lobe), epistaxis, hemoptysis, nasal mucosa swelling, oropharyngeal pain, pneumonitis, productive cough, pulmonary infection, respiratory failure, upper respiratory tract infection (CMV)

Miscellaneous: Laceration

Warnings/Precautions

Concerns related to adverse effects:

• Antibody formation: Development of antibodies may occur. In patients who experience a persistent decrease in preinjection levels of plasma adenosine deaminase (ADA) to <15 mmol/hour/L and have had other causes ruled out (eg, noncompliance), antibody formation should be considered and a specific assay for antibody ADA should be performed. Dosage adjustments may be required in patients developing antibodies.

Disease-related concerns:

• Immune deficiency: Immune deficient patients are at increased risk of infection; maintain adequate precautions until improvement in immune function.

• Thrombocytopenia: Use with caution in patients with thrombocytopenia due to risk of injection site bleeding; avoid use in patients with severe thrombocytopenia.

Monitoring Parameters

Trough plasma adenosine deaminase (ADA) activity; trough plasma deoxyadenosine nucleotides (dAXP) levels; total lymphocyte counts. More frequent monitoring may be necessary if therapy interrupted or clearance increases. Blood samples for analysis should be collected prior to the first administration for the week.

Plasma ADA activity (trough preinjection): Every 2 weeks (pegademase bovine naive patients) and every 4 weeks (patients transitioning from pegademase bovine) for the first 8 to 12 weeks of therapy, then every 3 to 6 months. Perform testing for antibodies to elapegademase if persistent decrease in levels below 15 mmol/hour/L occurs. Monitor immune function and clinical response closely and take precautions to minimize infection if a persistent decrease in ADA activity occurs.

Erythrocyte dAXP levels (trough preinjection): After 2 months, then a minimum of twice yearly

Lymphocyte counts (total and subset): Every 4 to 8 weeks for up to 1 year, then every 3 to 6 months (pegademase bovine naive patients) or every 3 to 6 months (all other patients)

Pregnancy Considerations

Animal reproduction studies have not been conducted.

If treatment with elapegademase is needed during pregnancy, close monitoring is recommended.

Patient Education

What is this drug used for?

• It is used to treat severe immune deficiency due to adenosine deaminase deficiency.

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

• Cough

• Vomiting

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

• Infection

• Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.

Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.

Consumer Information Use and Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a limited summary of general information about the medicine's uses from the patient education leaflet and is not intended to be comprehensive. This limited summary does NOT include all information available about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not intended to provide medical advice, diagnosis or treatment and does not replace information you receive from the healthcare provider. For a more detailed summary of information about the risks and benefits of using this medicine, please speak with your healthcare provider and review the entire patient education leaflet.

Further information

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

Frequently Asked Questions