57665-0002-XX REVCOVI 2.4MG/1.5ML Solution (LEADIANT BIOSCIENCES)
Elapegademase-lvlr is a recombinant adenosine deaminase (rADA) that is covalently conjugated to monomethoxypolyethylene glycol (mPEG) to produce methoxypolyethylene glycol recombinant adenosine deaminase (SC-PEG rADA), or Revcovi™. It is based on bovine amino acid sequence.
The deficiency of adenosine
deaminase (ADA) is associated with SCID, severe combined immune deficiency,
a rare, inherited and often fatal disease. ADA
enzyme is involved in purine metabolism as well as maintaining the proper
number and function of immune cells and decreasing the frequency of opportunistic
infections. Elevated adenosine levels, as occur in ADA deficiency, contribute
to apoptosis and a block in the differentiation of thymocytes, causing
Elapegademase-lvlr provides an exogenous source of ADA enzyme that is associated with a decrease in toxic adenosine and deoxyadenosine nucleotides levels as well as an increase in lymphocyte number.
Elapegademase-lvlr for the treatment of adenosine deaminase (ADA) deficiency is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Elapegademase-lvlr for the treatment of other conditions/diseases is considered investigational.
Must not be used in combination with pegademase-bovine; AND
Patient does not have severe thrombocytopenia (<50,000/microL); AND
Adenosine Deaminase (ADA) deficiency
Patient has severe combined immunodeficiency disease (SCID) with a definitive diagnosis of adenosine deaminase deficiency as determined by one of the following:
Deficient ADA catalytic activity (<1% of normal) in hemolysates (in untransfused individuals) or in extracts of other cells (e.g., blood mononuclear cells, fibroblasts); OR
Detection of biallelic pathogenic mutations in the ADA gene by molecular genetic testing; AND
Patient has a marked elevation of the metabolite deoxyadenosine triphosphate (dATP) or total deoxyadenosine nucleotides (dAXP) in erythrocytes; AND
Patient is not a candidate for or has failed bone marrow transplantation (BMT); AND
Baseline values for trough plasma ADA activity, red blood cell deoxyadenosine triphosphate (dATP), trough deoxyadenosine nucleotide (dAXP) and/or total lymphocyte counts have been obtained
Patient continues to meet universal and other indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in Initial Approval Criteria; AND
Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: injection site bleeding in patients with thrombocytopenia, severe thrombocytopenia, etc.; AND
Adequate documentation of disease stability and/or improvement as indicated by one or more of the following:
Increase in plasma ADA activity (target trough level ≥ 15 mmol/hr/L)
Red blood cell dATP level decreased (target ≤ 0.005 to 0.015 mmol/L)
Improvement in immune function with diminished frequency/complications of infection as evidenced in improvement in the ability to produce antibodies
Improvement in red blood cell dAXP levels (target trough level ≤ 0.02 mmol/L)
Patients transitioning from Adagen to Revcovi:
· If a patient’s weekly Adagen dose is unknown, or a patient’s weekly Adagen dose is at or lower than 30 U/kg, the recommended minimum starting dose of Revcovi is 0.2 mg/kg, intramuscularly, once a week
· If a patient’s weekly Adagen dose is above 30 U/kg, an equivalent weekly Revcovi dose (mg/kg) should be calculated using the following conversion formula:
Revcovi dose in mg/kg = Adagen dose in U/kg ÷ 150
· Subsequent doses may be increased by increments of 0.033 mg/kg weekly if trough ADA activity is under 30 mmol/hr/L, trough deoxyadenosine nucleotides (dAXP) are above 0.02 mmol/L, and/or the immune reconstitution is inadequate based on the clinical assessment of the patient. The total weekly dose may be divided into multiple intramuscular (IM) administrations during a week.
· The starting weekly dose of Revcovi is 0.4 mg/kg based on ideal body weight§ or actual weight (whichever is greater), divided into two doses (0.2 mg/kg twice a week), intramuscularly, for a minimum of 12 to 24 weeks until immune reconstitution is achieved.
· The dose may be gradually adjusted down to maintain trough ADA activity over 30 mmol/hr/L, trough dAXP level under 0.02 mmol/L, and/or to maintain adequate immune reconstitution based on clinical assessment of the patient.
§The Devine formula for ideal body weight:
· Ideal body weight (men) = 50 kg + 2.3 kg x ( height, in - 60 )
· Ideal body weight (women) = 45.5 kg + 2.3 kg x ( height, in - 60 )
· Note: this formula is only an approximation, and is generally only applicable for people 60 inches (5 foot) tall or greater. For patients under 5 feet, one commonly-used modification is to subtract 2-5 lbs for each inch below 60 inches (Devine BJ. Gentamicin therapy. Drug Intell Clin Pharm.1974;8:650–655.)
LENGTH OF AUTHORIZATION
Coverage will be provided for 12 months and may be renewed.
Max Units (per dose and over time) [HCPCS Unit]:
23 mg twice weekly
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).
1. Revcovi [package insert]. Indianapolis, IN; Leadiant Biosciences; December 2020. Accessed January 2021.
2. Hershfield, M. Adenosine Deaminase Deficiency. GeneReviews. www.ncbi.nlm.nih.gov/books/NBK1483/. Initial Posting: October 3, 2006; Last Update: March 16, 2017. Accessed January 2020.
3. Gaspar HB, Aiuti A, Porta F, et al. How I treat ADA deficiency. Blood. 2009 October 22; 114(17): 3524–3532.
4. Adenosine Deaminase Deficiency-genetic and Rare Diseases Information Center. US Department of health and human services-NIH. Available at: https://rarediseases.info.nih.gov/diseases/5748/adenosine-deaminase-deficiency
5. Flinn AM, Gennery AR. Adenosine deaminase deficiency: a review. Orphanet Journal of Rare Diseases 2018. https://doi.org/10.1186/s13023-018-0807-5
6. Lexicomp Online. (2021). AHFS DI. Elapegademase-lvlr. Retrieved February 11, 2021 from Lexicomp Online with AHFS.
7. MICROMEDEX Healthcare Series. Drugdex Drug Evaluations. (2019, December). Elapegademase-lvlr. Retrieved February 14, 2020 from MICROMEDEX Healthcare Series.
ORIGINAL EFFECTIVE DATE: 3/2/2019
MOST RECENT REVIEW DATE: 3/9/2021
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