Revcovi (elapegademase-lvlr)

POLICY NUMBER

L.5.01.619

Revcovi (elapegademase-lvlr)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Adenosine deaminase (ADA) deficiency is an autosomal recessive genetic disorder. The absence of functional ADA causes an intracellular accumulation of adenosine and deoxyadenosine, which are toxic to lymphocytes. This leads to a dysfunction of T, B, and natural killer (NK) cells and typically results in severe combined immunodeficiency (SCID). Unless identified on a newborn screening, symptoms usually present in the first few months of life and may include failure to thrive and life-threatening infections. Treatment should begin as soon as the diagnosis is established. Enzyme replacement treatment (ERT) is recommended to maintain a nontoxic metabolic environment; this helps stabilize the clinical status, restore immune function, and may prevent tissue/organ damage. Definitive therapy includes gene therapy and hematopoietic cell transplantation.

Revcovi (elapegademase-lvlr) is a recombinant ADA that is indicated for the treatment of ADA-SCID in pediatric and adult patients.

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. 

Initial Criteria

Revcovi (elapagademase-lvlr) may be considered medically necessary when ALL of the following criteria are met:

1. The individual has a documented diagnosis of adenosine deaminase severe combined immune deficiency (ADA-SCID) confirmed by ONE of the following:

   1. The individual has had absent or very low (<1% of normal) adenosine deaminase (ADA) catalytic activity; OR

   2. The individual has had molecular genetic testing confirming bi-allelic mutations in the ADA gene;

2. ONE of the following:

   1. The individual has NOT undergone curative treatment with hematopoietic cell transplant (HCT) or gene therapy; OR

   2. Curative therapy has been unsuccessful;

3. The individual does not have severe thrombocytopenia (i.e., platelet count <50×10⁹/L);

4. The requested agent is prescribed by, or in consultation with, an immunologist, hematologist/oncologist, or physician who specializes in the treatment of ADA-SCID or related disorder;

5. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Renewal Evaluation

1. The individual has been previously approved for the requested agent through the BCBSMS PA process;

2. The individual has demonstrated a beneficial response to therapy compared to pretreatment baseline (i.e., increase or stabilized plasma ADA activity, improvement in immune function, increase in total lymphocyte counts)

3. ONE of the following:

   1. The individual has NOT undergone curative treatment with hematopoietic cell transplant (HCT) or gene therapy; OR

   2. Curative therapy has been unsuccessful;

4. The individual does not have severe thrombocytopenia (i.e., platelet count <50×10⁹/L);

5. The requested agent is prescribed by, or in consultation with, an immunologist, hematologist/oncologist, or physician who specializes in the treatment of ADA-SCID or related disorder;

6. The individual does not have any FDA-labeled contraindication(s) to therapy with the requested agent; AND

7. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: 12 months

Services related to delivery and/or administration of a medication which have not been approved through the BCBSMS PA review process will be considered not medically necessary.

POLICY EXCEPTIONS

State Health Plan (State and School Employees) Participants

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the individual’s specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat an individual’s illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A.  consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B.  appropriate with regard to standards of good medical practice; and

C.  not solely for the convenience of the Member, his or her Provider; and

D.  the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

BCBSMS may request medical records for determination of medical necessity. When medical records are requested, letters of support and/or explanation are often useful, but are not sufficient documentation unless all specific information needed to make a medical necessity determination is included.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors.  In order for equipment, devices, drugs or supplies [i.e., technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

09/01/2025: New policy added.

SOURCE(S)

1. Revcovi prescribing information. Chiesi USA, Inc. August 2022. Last accessed June 2025.

2. Adenosine deaminase deficiency: Treatment and prognosis. UpToDate. Updated July 2023. Last accessed June 2025.

   https://www.uptodate.com/contents/adenosine-deaminase-deficiency-treatment-and-prognosis

3. Kohn DB, et al. Consensus approach for the management of severe combined immune deficiency caused by adenosine deaminase deficiency. J Allergy Clin Immunol. March 2019. 143(3):852-863. doi: 10.1016/j.jaci.2018.08.024

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

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