CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

RevCovi (elapegademase-lvlr) (Intramuscular) — Coverage Criteria for ADA‑SCID

Clinical coverage criteria for use, dosing, authorization and renewal of RevCovi for members (Medicaid, Commercial, Medicare-Medicaid Plan) diagnosed with adenosine deaminase severe combined immunodeficiency (ADA-SCID).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyRevCovi (elapegademase-lvlr) (Intramuscular) — Coverage Criteria for ADA‑SCID

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateSep 1, 2019

Next Review DateN/A

Key ActionSubmit prior authorization with documented diagnostic confirmation (ADA activity <1% or biallelic ADA mutation), baseline and ongoing labs (trough ADA activity, dATP/dAXP, lymphocyte counts), and statement on bone marrow transplant candidacy or use as bridge therapy.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12Initial authorization length

20NDC quantity limit (vials per 7 days)

23Maximum HCPCS units (per dosing guidance)

>=15Target trough plasma ADA activity (renewal target)

D81.31

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Covered diagnosis code (ADA deficiency with SCID)

RevCovi (elapegademase-lvlr) Coverage Criteria

Universal Criteria

All of the following must be met:

ALL of the following

Patient does not have severe thrombocytopeniaplatelet count >= 50,000/microL
Severe thrombocytopenia defined as platelet count <50,000/microL is exclusionary.

Initial Approval Criteria — ADA-SCID

Coverage provided when ALL of the following are met:

ALL of the following

Patient has adenosine deaminase severe combined immunodeficiency (ADA-SCID)
Diagnosis confirmation (one required)
- Deficient ADA catalytic activity (<1% of normal) in hemolysates (in untransfused individuals) or in extracts of other cells (e.g., blood mononuclear cells, fibroblasts)
- Detection of biallelic pathogenic mutations in the ADA gene by molecular genetic testing
Patient has elevated deoxyadenosine triphosphate (dATP) or total deoxyadenosine nucleotides (dAXP) in red blood cells
Definitive therapy / bridge therapy status (one required)
- Patient is not a candidate for or has failed definitive therapy with bone marrow transplantation (BMT)
- Patient is a candidate for definitive therapy with BMT and Revcovi (elapegademase) will be used as bridge therapy
Patient has baseline laboratory values for trough plasma ADA activity, red blood cell (dATP), trough red blood cell dAXP and/or total lymphocyte counts

Renewal Criteria

Coverage may be renewed when ALL of the following are met:

ALL of the following

Patient continues to meet universal and indication-specific criteria (including concomitant therapy requirements, performance status, etc.)
Absence of unacceptable toxicity from the drug
Examples: injection site bleeding in thrombocytopenic patients, severe thrombocytopenia, delay in improvement of immune function
Demonstrated beneficial response to therapy (one or more required)
- Increase in plasma ADA activitytarget trough level >= 15 mmol/hr/L
- Decrease in red blood cell dATP level

Relevant Codes and Billing

Covered ICD-10 Diagnosis CodesICD-10Covered

D81.31

Adenosine deaminase (ADA) deficiency with severe combined immunodeficiency

HCPCS / Billing CodesHCPCS

J3590	Unclassified biologics
C9399	Unclassified drugs or biologicals (Hospital Outpatient Use ONLY)

NDCNDC

57665-0002-xx

Revcovi 2.4 mg/1.5 mL single-dose vial

Prior Authorization, Documentation, Dosing, and Denials

Prior Authorization

Prior authorization required with documented diagnostics and labs

Prior authorization is required. Submit documentation confirming ADA-SCID diagnosis (deficient ADA catalytic activity <1% or biallelic pathogenic ADA gene mutations), evidence of elevated dATP/dAXP, baseline labs (trough plasma ADA activity, red blood cell dATP and dAXP, total lymphocyte counts), and a statement whether the patient is not a candidate for BMT or will use Revcovi as bridge therapy.

Affected codes: D81.31; HCPCS: J3590, C9399

Documentation Required

Baseline and ongoing laboratory monitoring

Provide baseline trough plasma ADA activity, red blood cell dATP and dAXP, and total lymphocyte counts with the initial request; submit ongoing laboratory results (e.g., trough ADA, dATP/dAXP, lymphocyte counts) to support renewals and demonstrate response.

Clinical Background

RevCovi (elapegademase-lvlr) is a pegylated recombinant adenosine deaminase indicated for the treatment of Adenosine Deaminase Severe Combined Immunodeficiency (ADA‑SCID). Clinical trial evidence demonstrates that RevCovi improves immune function—shown by increases in lymphocyte counts and normalization of T‑cell receptor excision circle (TREC) levels—and is associated with reductions in serious infections and hospitalizations compared with historical controls, indicating meaningful clinical benefit for patients with this condition.

Common adverse events reported with RevCovi therapy include injection‑site reactions, fever, and rash. These safety findings are balanced against the drug’s demonstrated ability to restore immune detoxification pathways and decrease infection‑related morbidity in ADA‑SCID patients.

Defined Terms

Definitions

ADA-SCIDAdenosine deaminase severe combined immunodeficiency, a genetic disorder causing profound T-, B-, and NK-cell lymphopenia due to ADA deficiency.

dAXPTotal deoxyadenosine nucleotides in red blood cells (biomarker of detoxification) including dATP.

Trough plasma ADA activityMeasured residual ADA enzymatic activity in plasma prior to next dose; used to assess biochemical response.

Bridge therapyTemporary therapy used to stabilize a patient prior to definitive treatment such as bone marrow transplantation.

Clinical Thresholds, Dosing, and Quantity Limits

Key Thresholds

Platelet count>= 50,000/microL (counts below exclude therapy)

Trough plasma ADA activity (renewal target)>= 15 mmol/hr/L

Red blood cell dATP (renewal target)<= 0.005 to 0.015 mmol/L

Red blood cell dAXP (renewal target)<= 0.02 mmol/L

Starting dose (Adagen-naïve)0.4 mg/kg/week divided into 0.2 mg/kg twice weekly (minimum 12–24 weeks)

Starting dose (transition from Adagen if unknown or <=30 U/kg)0.2 mg/kg IM once weekly

Conversion formula (Adagen >30 U/kg)

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyRevCovi (elapegademase-lvlr) (Intramuscular) — Coverage Criteria for ADA‑SCID

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateSep 1, 2019

Next Review DateN/A

SourceLink

On This Page

target <= 0.005 to 0.015 mmol/L

Improvement in immune function with diminished frequency/complications of infection (e.g., improved ability to produce antibodies)

Decrease in red blood cell dAXP levelstarget trough level <= 0.02 mmol/L

Increase in total lymphocyte counts

Billing Rule

Dose calculation and adjustment

When transitioning from Adagen, calculate the equivalent Revcovi dose using the conversion formula or specified starting doses; adjust dose in 0.033 mg/kg weekly increments based on biochemical and clinical indicators.

Example adjustment triggers: trough ADA activity <30 mmol/hr/L
dAXP >0.02 mmol/L
Inadequate clinical immune reconstitution

Denial Risk

Denial risk for severe thrombocytopenia or missing diagnostics

Requests will be denied if the member has severe thrombocytopenia (platelets <50,000/microL) or if required diagnostic/laboratory confirmation is not provided with the request.

Note

Authorization duration and renewals

Initial authorization is provided for 12 months. Renewals may be granted when renewal criteria are met, including absence of unacceptable toxicity and demonstration of biochemical or clinical benefit per policy targets.

Initial coverage 12 months; renewals per criteria

Revcovi mg/kg = Adagen U/kg ÷ 150

Max NDC quantity limit20 vials per 7 days (Revcovi 2.4 mg/1.5 mL vial)

Max HCPCS units23 mg twice weekly

12Initial authorization length (months)

20NDC quantity limit (vials per 7 days)

23Maximum HCPCS units (mg twice weekly)

>=15Target trough plasma ADA activity (mmol/hr/L)