BlueCross BlueShield of Tennessee Medical Policy Manual

Elapegademase-lvir (Revcovi®)

NDC CODE(S)

57665-0002-XX REVCOVI 2.4MG/1.5ML Solution (LEADIANT BIOSCIENCES)

DESCRIPTION

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 severe T-lymphopenia. 

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.

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

Universal Criteria

Adenosine Deaminase (ADA) deficiency

RENEWAL CRITERIA

DOSAGE/ADMINISTRATION

INDICATION

DOSE

Adenosine

deaminase

deficiency

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.

 

Adagen-naïve patients:

·         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.

DOSAGE LIMITS

Max Units (per dose and over time) [HCPCS Unit]:

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.

IMPORTANT REMINDER

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.

ADDITIONAL INFORMATION  

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).

SOURCES

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

ID_MRx

Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.

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