Version May 2024
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.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Elapegademase: Drug information")
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.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
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
There are no contraindications listed in the manufacturer's labeling.
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.
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)
No
Solution (Revcovi Intramuscular)
2.4 mg/1.5 mL (per mL): $10,202.00
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Parenteral: IM: For IM administration only; do not administer IV or SubQ; remove vial from refrigerator and allow to reach room temperature for 30 minutes; using polypropylene syringe and a ≥25-gauge needle withdraw dose from vial; prior to administration, change needle to a gauge and length appropriate for the patient's age and anatomy; administer immediately and without further dilution; discard of any unused medication.
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.
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.
Treatment of adenosine deaminase severe combined immune deficiency (ADA-SCID) (FDA approved in pediatric patients [age not specified] and adults).
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Pegloticase: May diminish the therapeutic effect of PEGylated Drug Products. Risk C: 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. Risk C: Monitor therapy
Animal reproduction studies have not been conducted.
If treatment with elapegademase is needed during pregnancy, close monitoring is recommended.
Baseline: Trough plasma adenosine deaminase (ADA) activity and trough erythrocyte deoxyadenosine nucleotides (dAXP) levels.
Following treatment initiation: Varies based on previous therapies.
Pegademase bovine (Adagen)-naive:
Plasma ADA activity (trough): Every 2 weeks during first 8 to 12 weeks of treatment, then every 3 to 6 months thereafter; perform testing for antibodies to elapegademase if persistent decrease in levels below 15 mmol/hour/L occurs. Monitor immune function and clinical status closely and take precautions to minimize infection if a persistent decrease in ADA activity occurs.
Erythrocyte dAXP levels (trough): Two months after initiation and then at least twice annually.
Total and subset lymphocytes (immune function): Every 4 to 8 weeks for up to a year after initiation and then every 3 to 6 months thereafter.
Patients converted from pegademase bovine (Adagen) to elapegademase (Revcovi):
Plasma ADA activity (trough): Every 4 weeks during first 8 to 12 weeks of treatment, then every 3 to 6 months thereafter.
Erythrocyte dAXP concentrations (trough): Two months after initiation and then at least twice annually.
Total and subset lymphocytes (immune function): Every 3 to 6 months.
Target concentrations:
Plasma ADA activity: Trough: ≥30 mmol/hour/L.
Erythrocyte dAXP concentration: Trough: <0.02 mmol/L.
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
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