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Asparaginase Erwinia Chrysanthemi (recombinant)-rywn (Rylaze®)
MA08.085i

Policy

The Company reserves the right to reimburse only those services that are furnished in the most appropriate and cost-effective setting that is appropriate to the member's medical needs and condition.

MEDICALLY NECESSARY ​​
​
​ASPARAGINASE​ ERWINIA CHRISANTHEMI (RECOMBINANT)-RYWN (RYLAZE)
Acute Lymphoblastic Leukemia (ALL) and Lymphoblastic Lymphoma (LBL) 

Asparaginase erwinia​ chrysanthemi (recombinant)-rywn (Rylaze) is considered medically necessary and, therefore, covered for the treatment of ALL and LBL in adult and pediatric individuals 1 month or older when all of the following criteria are met:
  • Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze) is being used as a component of a multiagent chemotherapeutic regimen 
  • The individual has a history of documented hypersensitivity to Escherichia coli (E. coli)–derived asparaginase (e.g., calaspargase pegol-mknl [Asparlas], pegaspargase [Oncaspar]) (and has fully recovered), or has silent inactivation
  • The individual does not have any of the following​ conditions/contraindications:
    • History of serious hypersensitivity reactions to asparaginase erwinia chrysanthemi (Erwinaze), including anaphylaxis 
    • History of Grade 3​ or higher pancreatitis with prior L-asparaginase therapy 
    • ​History of asparaginase-associated Grade 3​ or higher hemorrhagic event or asparaginase-associated thrombus requiring anticoagulation therapy, excluding catheter-related thrombotic events with prior L-asparaginase therapy 
    • History of severe hepatic impairment
Extranodal natural killer (NK)/T-cell lymphomas    

​Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze) is considered medically necessary and, therefore, covered for the treatment of extranodal NK/T-cell lymphomas when all of the following criteria are met:
  • Asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ is being used as a component of a multiagent chemotherapeutic regimen 
  • The individual has a history of documented hypersensitivity to pegaspargase [Oncaspar]) 
  • The individual does not have any of the following contraindications:
    • History of serious hypersensitivity reactions to asparaginase Erwinia chrysanthemi (Erwinaze), including anaphylaxis 
    • History of Grade 3 or higher pancreatitis with prior L-asparaginase therapy 
    • History of asparaginase-associated Grade 3 or higher hemorrhagic event or asparaginase-associated thrombus requiring anticoagulation therapy, excluding catheter-related thrombotic events with prior L-asparaginase therapy 
    • History of severe hepatic impairment​
ADDITIONAL INDICATIONS FOR ASPARAGINASE​ ERWINIA CHRISANTHEMI (RECOMBINANT)-RYWN (RYLAZE)
​ 
In accordance with the Centers for Medicare & Medicaid Services (CMS) and in addition to the indications above, asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ is covered for the following Micromedex Category IIb indications:
  • Acute myeloid leukemia
  • Chronic myeloid leukemia
  • Malignant lymphoma​​
EXPERIMENTAL/INVESTIGATIONAL

All other uses for asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) are considered experimental/investigational and, therefore, not covered unless the indication is supported as an accepted off-label use, as defined in the Company medical policy on off-label coverage for prescription drugs and biologics.

REQUIRED DOCUMENTATION

The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the professional provider's office, hospital, nursing home, home health agencies, therapies, and test reports.

The Company may conduct reviews and audits of services to our members, regardless of the participation status of the provider. All documentation is to be available to the Company upon request. Failure to produce the requested information may result in a denial for the drug.

Guidelines

There is no Medicare coverage determination addressing asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​; therefore, the Company policy is applicable.

NATIONAL CANCER INSTITUTE COMMON TERMINOLOGY CRITERIA FOR ADVERSE EVENTS

The National Cancer Institute Common Terminology Criteria for Adverse Events describes the severity of adverse events (i.e., organ toxicity) for individuals receiving chemotherapy. Adverse events are evaluated on a Grade Scale of 1 to 5 with unique clinical descriptions of severity for each adverse event. The general guideline is:
  • Grade 1: Mild: asymptomatic or mild symptoms; clinical or diagnostic observations only; intervention not indicated
  • Grade 2: Moderate: minimal, local, or noninvasive intervention indicated; limiting age-appropriate instrumental activities of daily living (ADL)
  • Grade 3: Severe or medically significant but not immediately life-threatening: hospitalization or prolongation of hospitalization indicated; disabling; limiting self-care ADL
  • Grade 4: Life-threatening​ consequences; urgent intervention indicated
  • Grade 5: Death related to adverse event
For additional information about organ-specific clinical descriptions, refer to https://ctep.cancer.gov/protocoldevelopment/electronic_applications/ctc.htm.

BENEFIT APPLICATION

Subject to the applicable Evidence of Coverage, asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) is covered under the medical benefits of the Company’s Medicare Advantage products when the medical necessity criteria listed in this medical policy are met.

For Medicare Advantage members, certain drugs are available through either the member's medical benefit (Part B benefit) or pharmacy benefit (Part D benefit), depending on how the drug is prescribed, dispensed, or administered. This medical policy only addresses instances when asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ is covered under a member's medical benefit (Part B benefit). It does not address instances when asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​​ is covered under a member’s pharmacy benefit (Part D benefit).

US FOOD AND DRUG ADMINISTRATION (FDA) STATUS

Asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) was approved by the FDA on​ June 30, 2021, for the treatment of individuals with acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in adult and pediatric individuals aged 1 month or older who have developed hypersensitivity to Escherichia coli (E. coli)–derived asparaginase.

PEDIATRIC ​
The safety and effectiveness of asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) have been established in pediatric individuals aged 1 month and older as a component of a multiagent chemotherapeutic regimen for the treatment of individuals with ALL and LBL​ who have developed hypersensitivity to E. coli–derived asparaginase.​

Description

According to the American Cancer Society, there are approximately 6590 new cases of acute lymphoblastic leukemia (ALL) occurring in children and adults in the United States. Children younger than 5 years of age have the highest level of risk for developing ALL. ALL, also known as acute lymphocytic leukemia, is a cancer that starts in the white blood cell precursors in the bone marrow and progresses rapidly to spread to other parts of the body.

Treatment for ALL is typically divided into several phases: induction, consolidation, and maintenance. Induction therapy is the initial phase of treatment with the objective of achieving complete remission. Consolidation, or the intensification phase, is a shorter period of chemotherapy to further reduce the amount of leukemic cells and to prevent the development of drug resistance. The individual's level of risk will determine the intensity of the chemotherapy regimen. The maintenance phase helps to prolong the duration of remission.

Asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) is an enzyme that catalyzes the conversion of the amino acid L-asparagine into aspartic acid and ammonia. Cancer cells need a source of L-asparagine to survive and function. The cancer cells cannot produce L-asparagine on their own, so rely on an external source. Rylaze exhausts any supply of L-asparagine in the cancer cells which results in an anticancer effect through cell death. Asparaginase is commonly used for the treatment of ALL. Clinical studies have demonstrated that asparaginase can be used to achieve complete remission for individuals during their first relapse.

ASPARAGINASE​ ERWINIA CHRISANTHEMI [RECOMBINANT]-RYWN (RYLAZE) 

On June 30, 2021, asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ was approved by the US Food and Drug Administration (FDA) for use as a component of a multiagent chemotherapeutic regimen for the treatment of ALL or LBL in pediatric and adult individuals aged 1 month and older who have developed hypersensitivity to Escherichia coli (E. coli)–derived asparaginase. 

Asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) contains an asparagine-specific bacterial enzyme (L-asparaginase). L-asparaginase is a tetrameric enzyme that consists of four identical 35-kDa subunits with a combined molecular weight of 140 kDa. The amino acid sequence is identical to native asparaginase erwinia chrysanthemi (also known as crisantaspase). The activity of asparaginase erwinia chrysanthemi (recombinant)-rywn is expressed in units, defined as the amount of enzyme that catalyzes the conversion of 1 μmol of L-asparagine per reaction minute, per mg of protein. Asparaginase erwinia chrysanthemi (recombinant)-rywn is produced by fermentation of a genetically engineered Pseudomonas fluorescens bacterium containing the DNA that encodes for asparaginase erwinia chrysanthemi.

The safety of asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) was evaluated in a cohort of 33 individuals from ​the study JZP458-201 (NCT04145531) who received asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) 25 mg/m2 intramuscularly on Monday, Wednesday, and Friday for six doses as a replacement for a single dose of pegaspargase (Oncaspar) as a component of multiagent chemotherapy. The individuals had a median age of 11 years (range, 1 to 24 years); the majority of individuals were male (51 percent) and white (73 percent). The individuals received a median of four courses of asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ (range, 1 to 14 cycles); 48 percent of individuals received at least four courses.  

 The safety and effectiveness of asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze)​ in the treatment of ALL and LBL have been established in pediatric individuals aged 1 month to less than 17 years who have developed hypersensitivity to a long-acting E. coli–derived asparaginase. The trial included 84 pediatric individuals, including two infants (1 month to <2 years), 62 children (2 years to <12 years old), and 20 adolescents (12 years to <17 years old). The safety and effectiveness of asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) have not been established in pediatric individuals younger than 1 month of age. The most frequent serious adverse reactions (in 5 percent or greater of individuals) were febrile neutropenia, dehydration, pyrexia, stomatitis, diarrhea, drug hypersensitivity, infection, nausea, and viral infection. Permanent discontinuation due to an adverse reaction occurred in 9 percent of individuals who received the asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze) 25 mg/m2 dosage. Adverse reactions resulting in permanent discontinuation included hypersensitivity (6 percent) and infection (3 percent).​​

OFF-LABEL INDICATIONS  

There may be additional indications contained in the Policy section of this document due to evaluation of criteria highlighted in the Company’s off-label policy, and/or review of clinical guidelines issued by leading professional organizations and government entities.

References

American Hospital Formulary Service (AHFS). Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). AHFS Drug Information 2024. [UpToDate Lexidrug Web site]. 10/02/2024. Available at: https://online.lexi.com/lco/action/home [via subscription only]. Accessed October 8, 2024.

Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). [prescribing information]. Palo Alto, CA: Jazz Pharmaceuticals, Inc. 04/2024. Available at: https://www.rylazepro.com/. Accessed October 8, 2024.​ ​

Centers for Medicare & Medicaid Services (CMS). Medicare Benefit Policy Manual. Chapter 15: Covered medical and other health services. 50.4.5 - Off-Label Use of Drugs and Biologicals in an Anti-Cancer Chemotherapeutic Regimen (Rev. 212, 02-10-16). [CMS Web site]. Available at: https://www.cms.gov/Regulations-and-Guidance/Guidance/Manuals/Internet-Only-Manuals-Ioms-Items/Cms012673.html. Accessed October 8, 2024.

ClinicalTrials.gov. An open-label study of JZP-458 (RC-P) in patients with acute lymphoblastic leukemia (ALL)/lymphoblastic lymphoma (LBL). ClinicalTrials.gov Identifier: NCT04145531. First Posted: October 30, 2019; Last Update Posted: November 18, 2023. Available at: https://clinicaltrials.gov/. Accessed October 8, 2024.​ ​

Einsiedel HG, von Stackelberg A, Hartmann R, et. al. Long-term outcome in children with relapsed ALL by risk-stratified salvage therapy: results of trial acute lymphoblastic leukemia-relapse study of the Berlin-Frankfurt-Munster Group 87. J Clin Oncol. 2005;23(31):7942-7950.

Elsevier's Clinical Pharmacology Compendium. Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). [Clinical Key Web site]. 09/10/2024. Available at: https://www.clinicalkey.com/#!/ [via subscription only]. Accessed October 8, 2024.​

​Jaccard A, Gachard N, Marin B, et al. Efficacy of L-asparaginase with methotrexate and dexamethasone (AspaMetDex regimen) in patients with refractory or relapsing extranodal NK/T-cell lymphoma, a phase 2 study. Blood. 2011;117(6):1834-1839.

Keating GM. Asparaginase Erwinia chrysanthemi (Erwinase®): a guide to its use in acute lymphoblastic leukemia in the USA. BioDrugs. 2013;27(4):413-418.

Maese L, Loh ML, Choi MR, et al. Recombinant Erwinia asparaginase (JZP458) in acute lymphoblastic leukemia: results from the phase 2/3 AALL1931 study. Blood. 2023;141(7):704-712.

Merative Micromedex® DRUGDEX® (electronic version). Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). [Micromedex Web site]. 04/26/2024. Available at: https://www.micromedexsolutions.com/micromedex2/librarian [via subscription only]. Accessed October 8, 2024.

National Comprehensive Cancer Network (NCCN). NCCN Clinical Practice Guidelines in Oncology®. Acute Lymphoblastic Leukemia V2.2024. [NCCN Web site]. 07/19/2024. Available at: https://www.nccn.org/professionals/physician_gls/pdf/all.pdf. Accessed October 8, 2024.​ ​

National Comprehensive Cancer Network (NCCN). NCCN Clinical Practice Guidelines in Oncology®. Pediatric Acute Lymphoblastic Leukemia V1.2025. [NCCN Web site]. 08/28/2024. Available at: https://www.nccn.org/professionals/physician_gls/pdf/ped_all.pdf. Accessed October 8, 2024.​ 

National Comprehensive Cancer Network (NCCN). NCCN Clinical Practice Guidelines in Oncology®. T-Cell Lymphomas V4.2024. [NCCN Web site]. 05/28/2024. Available at: https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf. Accessed October 8, 2024.​ ​

National Comprehensive Cancer Network (NCCN). NCCN Drugs & Biologics Compendium®. [NCCN Web site]. Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). Available at: https://www.nccn.org/professionals/drug_compendium/content/ [via subscription only]. Accessed October 8, 2024.

Ribera JM, Garcia O, Oriol A, et al. Feasibility and results of subtype-oriented protocols in older adults and fit elderly patients with acute lymphoblastic leukemia: results of three prospective parallel trials from the PETHEMA group. Leuk Res. 2016;41:12-20.

Salzer WL, Asselin B, Supko JG, et al. Erwinia asparaginase achieves therapeutic activity after pegaspargase allergy: a report from the Children's Oncology Group. Blood. 2013;122(4):507-514.

Seiter K. Acute lymphoblastic leukemia (ALL) treatment & management. [Medscape Web site]. 04/17/2023. Available at: http://emedicine.medscape.com/article/207631-treatment. Accessed October 8, 2024.​ ​​

UpToDate® LexidrugTM. Asparaginase Erwinia chrysanthemi (recombinant)-rywn (Rylaze®). [UpToDate Lexidrug Web site]. 08/22/2024. Available at: https://online.lexi.com/lco/action/home [via subscription only]. Accessed October 8, 2024.

US Department of Health and Human Services. Common terminology criteria for adverse events (CTCTAE). V5.0. 04/19/2021. Available at: https://ctep.cancer.gov/protocoldevelopment/electronic_applications/ctc.htm. Accessed October 8, 2024.

US Food and Drug Administration (FDA). Center for Drug Evaluation and Research. Asparaginase (Erwinia chrysanthemi [recombinant)-rywn])​ (Rylaze®). Prescribing information. [FDA Web site]. 04/15/2024. Available at: https://www.accessdata.fda.gov/scripts/cder/daf/index.cfm. Accessed October 8, 2024.​ ​

Yamaguchi M, Kwong YL, Kim WS, et al. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol. 2011;29(33):4410-4416.​

Coding

CPT Procedure Code Number(s)
N/A
ICD - 10 Procedure Code Number(s)
N/A
ICD - 10 Diagnosis Code Number(s)
C83.50 Lymphoblastic (diffuse) lymphoma, unspecified site
C83.51 Lymphoblastic (diffuse) lymphoma, lymph nodes of head, face, and neck
C83.52 Lymphoblastic (diffuse) lymphoma, intrathoracic lymph nodes
C83.53 Lymphoblastic (diffuse) lymphoma, intra-abdominal lymph nodes
C83.54 Lymphoblastic (diffuse) lymphoma, lymph nodes of axilla and upper limb
C83.55 Lymphoblastic (diffuse) lymphoma, lymph nodes of inguinal region and lower limb
C83.56 Lymphoblastic (diffuse) lymphoma, intrapelvic lymph nodes
C83.57 Lymphoblastic (diffuse) lymphoma, spleen
C83.58 Lymphoblastic (diffuse) lymphoma, lymph nodes of multiple sites
C83.59 Lymphoblastic (diffuse) lymphoma, extranodal and solid organ sites
C84.90 Mature T/NK-cell lymphomas, unspecified, unspecified site
C84.91 Mature T/NK-cell lymphomas, unspecified, lymph nodes of head, face, and neck
C84.92 Mature T/NK-cell lymphomas, unspecified, intrathoracic lymph nodes
C84.93 Mature T/Nk-cell lymphomas, unspecified, intra-abdominal lymph nodes
C84.94 Mature T/NK-cell lymphomas, unspecified, lymph nodes of axilla and upper limb
C84.95 Mature T/NK-cell lymphomas, unspecified, lymph nodes of inguinal region and lower limb
C84.96 Mature T/NK-cell lymphomas, unspecified, intrapelvic lymph nodes
C84.97 Mature T/NK-cell lymphomas, unspecified, spleen
C84.98 Mature T/NK-cell lymphomas, unspecified, lymph nodes of multiple sites
C84.99 Mature T/NK-cell lymphomas, unspecified, extranodal and solid organ sites
C84.9A Mature T/NK-cell lymphomas, unspecified, in remission
C84.Z0 Other mature T/NK-cell lymphomas, unspecified site
C84.Z1 Other mature T/NK-cell lymphomas, lymph nodes of head, face, and neck
C84.Z2 Other mature T/NK-cell lymphomas, intrathoracic lymph nodes
C84.Z3 Other mature T/NK-cell lymphomas, intra-abdominal lymph nodes
C84.Z4 Other mature T/NK-cell lymphomas, lymph nodes of axilla and upper limb
C84.Z5 Other mature T/NK-cell lymphomas, lymph nodes of inguinal region and lower limb
C84.Z6 Other mature T/NK-cell lymphomas, intrapelvic lymph nodes
C84.Z7 Other mature T/NK-cell lymphomas, spleen​​
C84.Z8 Other mature T/NK-cell lymphomas, lymph nodes of multiple sites
C84.Z9 Other mature T/NK-cell lymphomas, extranodal and solid organ sites​
​​C84.ZA Other mature T/NK-cell lymphomas, in remission​
C86.00 Extranodal NK/T-cell lymphoma, nasal type not having achieved remission
C86.01 Extranodal NK/T-cell lymphoma, nasal type, in remission
C91.00 Acute lymphoblastic leukemia not having achieved remission
C91.01 Acute lymphoblastic leukemia, in remission
C91.02 Acute lymphoblastic leukemia, in relapse
C92.00 Acute myeloblastic leukemia, not having achieved remission
C92.01 Acute myeloblastic leukemia, in remission
C92.02 Acute myeloblastic leukemia, in relapse
C92.10 Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission
C92.11 Chronic myeloid leukemia, BCR/ABL-positive, in remission
C92.12 Chronic myeloid leukemia, BCR/ABL-positive, in relapse

HCPCS Level II Code Number(s)

​J9021 Injection, asparaginase, recombinant, (Rylaze), 0.1 mg​

NOT ELIGIBLE FOR REIMBURSEMENT

​THE FOLLOWING CODE REPRESENTS ASPARAGINASE (ERWINAZE) WHICH IS NO LONGER MANUFACTURED AND HAS BEEN WITHDRAWN FROM THE MARKET:

​J9019 Injection, asparaginase (Erwinaze), 1,000 IU​
Revenue Code Number(s)
N/A



Coding and Billing Requirements

Policy History

Revisions From MA08.085i:
01/01/2025This version of the policy will become effective 01/01/2025.
​​
The following drug, its policy criteria, and associated ICD-10 codes have been deleted from this policy as the drug is no longer manufactured or available in the US:
  • Asparaginase erwinia chrysanthemi (Erwinaze®)
The following HCPCS code was revised to be not eligible for reimbursement:
  • ​J9010 Injection, asparaginase (Erwinaze), 1,000 IU​
Criteria for asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze®)​ have been revised ​in accordance with US Food and Drug Administration (FDA) labeling (04/15/2024) and National Comprehensive Cancer Network (NCCN) compendium (accessed 10/08/2024).​​

Revisions From MA08.085h:
10/01/2024This version of the policy will become effective 10/01/2024.

Inclusion of a policy in a Code Update memo does not imply that a full review of
the policy was completed at this time.​

The following ICD-10 codes have been added to this policy:
C84.9A   Mature T/NK-cell lymphomas, unspecified, in remission
C84.ZA  Other mature T/NK-cell lymphomas, in remission
C86.00   Extranodal NK/T-cell lymphoma, nasal type not having achieved remission
C86.01   Extranodal NK/T-cell lymphoma, nasal type, in remission

​The following ICD-10 code has been deleted from this policy:
​C86.0 ​​ Extranodal NK/T-cell lymphoma, nasal type

Revisions From MA08.085​g:
​01/01/202​4
​Effective 01/01/2024 this policy applies to New Jersey Medicare Advantage (MA) lines of business.​
06/19/2023​
This version of the policy will become effective 06/19/2023​.

This policy was updated to communicate the Company's coverage position for Erwinaze ​and Rylaze, in alignment with the National Comprehensive Cancer Network (NCCN) and the Centers for Medicare & Medicaid Services (CMS) requirements for Micromedex Drug Compendia (Category IIb indications). 

The NCCN recommends Rylaze for the treatment of extranodal NK/T-cell lymphoma (all types). 

The NCCN recommends Rylaze for the treatment of relapsed ALL or LBL, so the criteria that "The individual does not have any of the following ​conditions/contraindications: relapsed ALL or LBL​" was removed. 

​The following ICD-10 CM codes have been added to this policy for Rylaze:
​C84.90    Mature T/NK-cell lymphomas, unspecified, unspecified site
C84.91    Mature T/NK-cell lymphomas, unspecified, lymph nodes of head, face, and neck
C84.92    Mature T/NK-cell lymphomas, unspecified, intrathoracic lymph nodes
C84.93    Mature T/NK-cell lymphomas, unspecified, intra-abdominal lymph nodes
C84.94    Mature T/NK-cell lymphomas, unspecified, lymph nodes of axilla and upper limb
C84.95    Mature T/NK-cell lymphomas, unspecified, lymph nodes of inguinal region and lower limb
C84.96    Mature T/NK-cell lymphomas, unspecified, intrapelvic lymph nodes
C84.97    Mature T/NK-cell lymphomas, unspecified, spleen
C84.98    Mature T/NK-cell lymphomas, unspecified, lymph nodes of multiple sites
C84.99    Mature T/NK-cell lymphomas, unspecified, extranodal and solid organ sites
C84.Z0    Other mature T/NK-cell lymphomas, unspecified site
C84.Z1    Other mature T/NK-cell lymphomas, lymph nodes of head, face, and neck
C84.Z2    Other mature T/NK-cell lymphomas, intrathoracic lymph nodes
C84.Z3    Other mature T/NK-cell lymphomas, intra-abdominal lymph nodes
C84.Z4    Other mature T/NK-cell lymphomas, lymph nodes of axilla and upper limb
C84.Z5    Other mature T/NK-cell lymphomas, lymph nodes of inguinal region and lower limb
C84.Z6    Other mature T/NK-cell lymphomas, intrapelvic lymph nodes
C84.Z7    Other mature T/NK-cell lymphomas, spleen​​
C84.Z8    Other mature T/NK-cell lymphomas, lymph nodes of multiple sites
C84.Z9    Other mature T/NK-cell lymphomas, extranodal and solid organ sites​

Revisions From MA08.085​f:
08/15/2022
This version of the policy will become effective 08/15/2022​.​

This policy was updated to communicate the Company's coverage position for Erwinaze ​and Rylaze, in alignment with National Comprehensive Cancer Network (NCCN) and the clinical study criteria​.

Erwinaze induction therapy for Acute Lymphoblastic Leukemia (ALL)​​ now includes individuals with substantial comorbidities. 
Erwinaze information for ALL was updated regarding use with tyrosine kinase inhibitors (TKI). 
Rylaze​ coverage criteria was clarified for ALL and LBL, in accordance with the clinical study criteria. 
Rylaze is covered for Acute myeloid leukemia, Chronic myeloid leukemia, and Malignant lymphoma​, in accordance with the Centers for Medicare and Medicaid Services (CMS) requirements for Micromedex Drug Compendia (Category IIb indications). 

Revisions From MA08.085​e:
01/01/2022This policy has been identified for the HCPCS code update, effective 01/01/2022​.

The following HCPCS codes have been termed from this policy:
C9399 Unclassified drugs or biologics​
J3590 Unclassified biologics 

The following HCPCS code has been added to this policy:
J9021 Injection, asparaginase, recombinant, (rylaze), 0.1 mg

Revisions From​ MA08.085​d:​
09/13/2021​​​This version of the policy will become effective 09/13/2021​​

The policy has been updated to communicate changes based on National Comprehensive Cancer Network (NCCN) and Micromedex DrugDex compendia. Criteria have been revised for Erwinase® for the treatment of Acute Lymphoblastic Leukemia (ALL). New drug asparaginase erwinia chrysanthemi [recombinant]-rywn (Rylaze™) and indications were added for the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in adult and pediatric individuals

​The following ICD-10 CM codes have been added to this policy: 
C83.50 Lymphoblastic (diffuse) lymphoma, unspecified site
C83.51 Lymphoblastic (diffuse) lymphoma, lymph nodes of head, face, and neck
C83.52 Lymphoblastic (diffuse) lymphoma, intrathoracic lymph nodes
C83.53 Lymphoblastic (diffuse) lymphoma, intra-abdominal lymph nodes
C83.54 Lymphoblastic (diffuse) lymphoma, lymph nodes of axilla and upper limb
C83.55 Lymphoblastic (diffuse) lymphoma, lymph nodes of inguinal region and lower limb
C83.56 Lymphoblastic (diffuse) lymphoma, intrapelvic lymph nodes
C83.57 Lymphoblastic (diffuse) lymphoma, spleen
C83.58 Lymphoblastic (diffuse) lymphoma, lymph nodes of multiple sites
C83.59 Lymphoblastic (diffuse) lymphoma, extranodal and solid organ sites​ ​

Revisions From​ MA08.085c:
01/18/2021The policy has been updated to communicate changes based on National Comprehensive Cancer Network (NCCN) and Micromedex DrugDex compendia. Criteria have been revised to include use of Erwinase® as a component of a multi-agent chemotherapeutic regimen for the treatment of extranodal NK/T-cell lymphoma, nasal type. Medical Necessity coverage was added for the following oncologic conditions, in accordance with the Centers for Medicare and Medicaid Services (CMS) and Micromedex DrugDex compendia (Category IIb indications)
  • Acute myeloid leukemia
  • Chronic myeloid leukemia
  • Malignant lymphoma​
​The following ICD-10 CM codes have been added to this policy: 

C85.80 Other specified types of non-Hodgkin lymphoma, unspecified site

C85.81 Other specified types of non-Hodgkin lymphoma, lymph nodes of head, face, and neck 

C85.82 Other specified types of non-Hodgkin lymphoma, intrathoracic lymph nodes 

C85.83 Other specified types of non-Hodgkin lymphoma, intra-abdominal lymph nodes 

C85.84 Other specified types of non-Hodgkin lymphoma, lymph nodes of axilla and upper limb 

C85.85 Other specified types of non-Hodgkin lymphoma, lymph nodes of inguinal region and lower limb 

C85.86 Other specified types of non-Hodgkin lymphoma, intrapelvic lymph nodes 

C85.87 Other specified types of non-Hodgkin lymphoma, spleen 

C85.88 Other specified types of non-Hodgkin lymphoma, lymph nodes of multiple sites 

C85.89 Other specified types of non-Hodgkin lymphoma, extranodal and solid organ sites 

​C86.0 Extranodal NK/T-cell lymphoma, nasal type

C91.01  Acute lymphoblastic leukemia, in remission

​C92.00   Acute myeloblastic leukemia, not having achieved remission

C92.01   Acute myeloblastic leukemia, in remission

C92.02   Acute myeloblastic leukemia, in relapse

C92.10   Chronic myeloid leukemia, BCR/ABL-positive, not having achieved remission

C92.11   Chronic myeloid leukemia, BCR/ABL-positive, in remission

C92.12   Chronic myeloid leukemia, BCR/ABL-positive, in relapse​


Revisions From MA08.085b:
12/16/2019This version of the policy will become effective 12/16/2019.

The policy has been updated to communicate changes based on National Comprehensive Cancer Network (NCCN). Criteria have been revised to include use of Erwinase® as first-line therapy for adult individuals over 65 years of age and used in pediatric individuals with acute lymphoblastic leukemia.

Revisions From MA08.085a:
11/19/2018.This version of the policy will become effective 11/19/2018.

The policy has been updated to communicate changes based on National Comprehensive Cancer Network (NCCN).Criteria have been revised to include hypersensitivity to Oncaspar.

Revisions From MA08.085:
04/11/2018This policy has been reissued in accordance with the Company's annual Policy Confirmation Review track. The references were updated accordingly. The policy was updated to be consistent with current template wording and format. The adoptable source for this policy is FDA labeling (03/2016), Micromedex (11/2016), and AHFS (02/2018), NCCN compendium (03/2018), NCCN Acute Lymphoblastic Leukemia Guidelines V1.2018 (03/2018).
06/07/2017This policy has been reissued in accordance with the Company's annual review process.
01/01/2017The following new policy has been developed to communicate of the Company’s coverage criteria for asparaginase Erwinia chrysanthemi (Erwinaze®).

1/1/2025
1/2/2025
MA08.085
Medical Policy Bulletin
Medicare Advantage
No