CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Piasky (crovalimab-akkz) for Paroxysmal Nocturnal Hemoglobinuria (PNH)

Defines accepted indications, contraindications, exclusion criteria, authorization/continuation rules, coding, and applicable lines of business for Piasky (crovalimab-akkz), with emphasis on FDA-approved and evidence-supported uses for PNH.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyPiasky (crovalimab-akkz) for Paroxysmal Nocturnal Hemoglobinuria (PNH)

Policy CodePolicy N/A

Change TypeTemplate conversion; new policy

Effective Date

Next Review Date

Key ActionAll medication requests must be processed by Evolent; medications not authorized by Evolent may be deemed not approvable and not reimbursable.

SourceLink

POLICY UPDATE CHANGES

Converted to new Evolent guideline template and replaced prior UM ONC_1505 Piasky policy.

New policy created in August 2024.

1Covered Indications (PNH)

4Key Exclusion Criteria listed

1HCPCS J-code listed

Coverage Summary & Indications

Policy: Piasky (crovalimab-akkz) is covered with criteria for the treatment of paroxysmal nocturnal hemoglobinuria (PNH) in patients 13 years or older with body weight of at least 40 kg. Coverage requires adherence to the policy’s indication and exclusion rules and prior authorization processing by Evolent (J1307). The drug carries a US boxed warning for serious meningococcal infections and is available only through the PIASKY REMS program with REMS-related vaccination and monitoring requirements.

Indication - Paroxysmal Nocturnal Hemoglobinuria (PNH)

Covered when ALL of the following are met:

Initial Therapy for PNH

Diagnosis of paroxysmal nocturnal hemoglobinuria (PNH)
Minimum age: Member age at least 13 years>= 13 years
Minimum body weight: Body weight of at least 40 kg>= 40 kg

Continuation Therapy & Exemption Rules

Continuation therapy exemption

Continuation requests for a not-approvable medication shall be exempt provided ALL of the following are met:

No disease progression: The member has not experienced disease progression on the requested medication
Recency of use: The requested medication was used within the last year without a lapse of more than 30 days of having an active authorizationused within last 1 year with no lapse > 30 days
No new meds added: Additional medication(s) are not being added to the continuation request

Contraindications / Warnings

Not appropriate / contraindicated when ANY of the following apply:

Hypersensitivity: Serious hypersensitivity to crovalimab-akkz or any component of the formulation
Unresolved meningococcal infection: Initiation in patients with an unresolved serious Neisseria meningitidis infection
Vaccination/REMS noncompliance: Member unable or unwilling to comply with meningococcal vaccination and REMS requirements (PIASKY REMS related risk mitigation)

Exclusion Criteria (Not Covered / Not Approvable)

Exclusion Criteria (Not covered / Not approvable)

Use is excluded when ANY of the following apply:

Exclusion Criteria

Disease progression while taking Piasky (crovalimab-akkz)
Concurrent PNH therapies: Concurrent use with other therapies used to treat PNH (e.g., eculizumab, ravulizumab)
Single dose limit: Dosing exceeds single dose limit<= 1500 mg
Investigational / Off-label insufficient evidence: Off-label indication not supported by FDA labeling, CMS-recognized compendia, NCCN/ASCO guidelines, or acceptable peer-reviewed literatureOff-label requires FDA labeling or CMS compendia or NCCN/ASCO or acceptable peer-reviewed literature meeting CMS standards
Insufficient evidence defined by: clinical characteristics not represented in published evidence; therapy regimen not represented; outcomes not clinically meaningful (e.g., ASCO definitions: HR < 0.80 or OS/PFS benefit < 3 months); inappropriate experimental design; solely case reports or meeting abstracts without full peer-reviewed articles.

Sufficient evidence for off-label use requires support from FDA labeling, CMS-recognized compendia, NCCN/ASCO guidelines, or peer-reviewed literature that meets CMS standards. The policy excludes anecdotal or insufficient evidence such as case reports or meeting abstracts without full peer-reviewed articles and applies CMS criteria (e.g., representativeness of patient clinical characteristics, appropriate regimen representation, clinically meaningful outcomes, and appropriate study design) when determining whether off-label use is supported.

Prior Authorization, Documentation & Billing Rules

Prior Authorization

Prior Authorization Required

Evolent is responsible for processing all medication requests from network ordering providers. Medications not authorized by Evolent may be deemed as not approvable and therefore not reimbursable.

Affected code: J1307

Documentation Required

Evidence Support Requirement

The use of this drug must be supported by one of the following: FDA approved product labeling, CMS-approved compendia, National Comprehensive Cancer Network (NCCN), American Society of Clinical Oncology (ASCO) clinical guidelines, or peer-reviewed literature that meets the requirements of the CMS Medicare Benefit Policy Manual Chapter 15.

Denial Risk

Denial Risk: Concurrent Complement Inhibitors

Claims will be excluded/denied for concurrent use with other complement inhibitors used to treat PNH.

Concurrent agents: eculizumab
Concurrent agents: ravulizumab

Documentation Required

Meningococcal Vaccination & REMS Compliance

Complete or update meningococcal vaccination (serogroups A, C, W, Y, and B) at least 2 weeks prior to the first dose of Piasky (crovalimab-akkz) unless the risks of delaying therapy outweigh the risk of infection. Comply with PIASKY REMS requirements and monitor patients closely for early signs of meningococcal infection; evaluate immediately if infection is suspected.

PIASKY REMS restricted program — REMS enrollment and requirements apply
US Boxed Warning: Serious meningococcal infections

Clinical Evidence & References

Key sources supporting the PNH indication include the COMMODORE 2 Phase 3 trial (Röth A et al. 2024) and the Piasky prescribing information (Genentech 2024). The COMMODORE 2 randomized Phase 3 trial comparing crovalimab with eculizumab is cited as the pivotal clinical evidence supporting use in PNH and is referenced alongside the manufacturer’s prescribing information.

Background & Safety

Background: Accepted indications include FDA-approved use for PNH in adults and pediatric patients aged 13 years and older who weigh at least 40 kg; off-label uses may be accepted when supported by recognized compendia or acceptable peer-reviewed literature. There is a US boxed warning for serious meningococcal infections; complete or update meningococcal vaccination (serogroups A, C, W, Y, and B) at least 2 weeks prior to the first dose is recommended unless delay is more harmful. Crovalimab is available only through the PIASKY REMS, and patients must comply with REMS requirements and be monitored for meningococcal infection.

Definitions

Continuation exemption: Continuation requests for otherwise not-approvable medication are exempt when ALL of the following are met: the member has not experienced disease progression on the requested medication; the medication was used within the last year without a lapse of more than 30 days of having an active authorization; and no additional medication(s) are being added to the continuation request.

Sufficient evidence for off-label use: Evidence must be supported by FDA labeling, CMS-recognized compendia, NCCN/ASCO guidelines, or peer-reviewed literature meeting CMS Medicare Benefit Policy Manual Chapter 15 standards; anecdotal case reports and meeting abstracts without full peer-reviewed articles are excluded and clinical evidence must meet criteria for representativeness, regimen applicability, clinically meaningful outcomes, and appropriate study design.