CurrentCareSourcePolicy N/A

Enzyme Replacement Therapy: Revcovi (elapegademase-lvlr) — Coverage Criteria

Policy governs prior authorization and medical necessity criteria for Revcovi (elapegademase-lvlr) intramuscular injection for treatment of adenosine deaminase severe combined immunodeficiency (ADA-SCID) for pediatric and adult patients.

Policy Summary

PayerCareSource

PolicyEnzyme Replacement Therapy: Revcovi (elapegademase-lvlr) — Coverage Criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionObtain prior authorization and document absent or very low (<1% of normal) ADA catalytic activity or bi-allelic pathogenic ADA variants and that a specialist prescribed or consulted.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

0.4 mg/kgmaximum weekly dose (IM)

1 yearinitial approval

<1%diagnostic threshold

5–8 yrsavoid long-term ERT >

Coverage Criteria for Revcovi (elapegademase-lvlr)

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Initial FDA-Approved Indication

Covered when BOTH of the following are met:

FDA-Approved Indication: ADA-SCID: Approve for 1 year if the patient meets BOTH of the following (A and B)

A: Diagnostic confirmation: Patient has a diagnosis of ADA-SCID confirmed by ONE of the following: i) absent or very low (<1% of normal) ADA catalytic activity at baseline OR ii) molecular genetic testing confirming bi-allelic pathogenic variants in the ADA gene.ADA activity <1%

Include laboratory documentation or genetic test report.

B: Prescriber requirement: Medication is prescribed by or in consultation with an immunologist, hematologist/oncologist, or physician who specializes in ADA-SCID or related disorders.

Dosing and Duration

Dosing and duration conditions:

Dosing: Approve up to a maximum weekly dose of 0.4 mg/kg by the intramuscular route; doses may be divided into multiple injections as long as weekly cumulative maximum is not exceeded.0.4 mg/kg/week

Requests for doses outside this dosing will be reviewed case-by-case by a clinician.

Approval duration: Initial approval for 1 year; extended approvals allowed if patient continues to meet criteria and dosing; long-term ERT beyond 5-8 years should be avoided unless HSCT or gene therapy unavailable or ineffective.1 year initial; avoid >5-8 years

Clinical review required for extensions.

Key Clinical Thresholds & Dosing Metrics

ADA catalytic activity — < 1% of normal

Diagnostic thresholdAbsent or very low ADA catalytic activity defined as < 1% of normal

Laboratory evidenceBaseline measurement of ADA catalytic activity required before initiating ERT

Supporting testingElevated adenosine or deoxyadenosine (plasma/urine/dried blood spot) supports diagnosis

Follow-up useADA catalytic activity used to monitor response and guide maintenance dosing adjustments

Maximum weekly dose (IM) — 0.4 mg/kg

Maximum weekly intramuscular dose0.4 mg/kg per week (intramuscular route)

Dosing frequencyWeekly total may be divided into multiple intramuscular injections as long as cumulative weekly maximum not exceeded

Initial dosing (naive patients)Starting weekly dose of 0.4 mg/kg (may be divided; continued for 12–24 weeks until immune reconstitution)

Dose exceptionsRequests for doses outside this maximum are reviewed case-by-case by a clinician

Prior Authorization, Documentation, and Provider Guidance

Prior Authorization

Prior Authorization Required

Prior authorization is recommended for Revcovi; approve for 1 year when criteria are met. Requests for doses outside the policy dosing will be reviewed on a case-by-case basis by a clinician (Medical Director or Pharmacist). Because of the specialized skills required, Revcovi must be prescribed by or in consultation with an immunologist, hematologist/oncologist, or physician who specializes in ADA-SCID or related disorders.

Approve up to a maximum weekly dose of 0.4 mg/kg IM (may be divided into multiple injections) — requests above this will be considered case-by-case
Automation: None

Note

Role of ERT Relative to Curative Therapies

Consensus guidance recommends ERT (enzyme replacement therapy) as an immediate stabilizing bridge to curative therapy (HSCT or hematopoietic stem cell gene therapy) for most patients with ADA-SCID. The typical bridging duration is a few months up to approximately 2 years. Long-term continuous ERT is discouraged; use beyond 5–8 years should be avoided and reserved only when HSCT or gene therapy are not available or have failed. ERT may also be considered for later-onset phenotypes who are not candidates for curative therapies.

Documentation Required

Required Diagnostic and Prescriber Documentation

Documentation must include diagnostic confirmation of ADA-SCID and specialist involvement. Require either absent or very low (<1% of normal) ADA catalytic activity at baseline or molecular genetic confirmation of bi-allelic pathogenic variants in the ADA gene. Document evidence of prescribing or consultation with an immunologist, hematologist/oncologist, or other physician who specializes in ADA-SCID.

Denial Risk

General Denial Trigger

Coverage is not recommended for situations not explicitly listed in the Recommended Authorization Criteria. Criteria will be updated as new published data become available.

Clinical Background: ADA-SCID and Enzyme Replacement Therapy

Background: Adenosine deaminase severe combined immunodeficiency (ADA‑SCID) is an ultra‑rare, autosomal recessive disorder of purine metabolism that results in accumulation of deoxyadenosine and dATP and causes marked depletion and dysfunction of B, T, and NK lymphocytes. Typical presentation occurs in infancy with failure to thrive and severe, recurrent infections; untreated ADA‑SCID is usually fatal within 1–2 years. Enzyme replacement therapy with Revcovi (elapegademase‑lvlr) is indicated to stabilize patients and is commonly used as a bridge to curative treatments such as hematopoietic stem cell transplant or gene therapy.

Definitions and Diagnostic Criteria

Revcovi — Revcovi (elapegademase-lvlr)

Generic and brandRevcovi (elapegademase-lvlr)

Drug classRecombinant adenosine deaminase (enzyme replacement therapy)

IndicationTreatment of adenosine deaminase severe combined immunodeficiency (ADA-SCID) in pediatric and adult patients

RouteIntramuscular administration as described in prescribing information

ADA-SCID diagnostic criteria — ADA-SCID diagnosis

Core diagnostic criteriaDiagnosis demonstrated by absent or very low (< 1% of normal) ADA catalytic activity

Genetic confirmationMolecular genetic testing confirming bi-allelic pathogenic variants in the ADA gene can establish diagnosis

Recommended testing sequenceBiochemical demonstration of low ADA activity followed by genetic testing to confirm bi-allelic mutations

Documentation requirementLaboratory or genetic test reports should be included with authorization requests