CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Cablivi (caplacizumab-yhdp) — coverage criteria for acquired thrombotic thrombocytopenic purpura (aTTP)

Defines medical necessity, inclusion and exclusion criteria, and utilization management requirements for coverage of Cablivi (caplacizumab-yhdp) for members (primarily for aTTP) processed by the payer's UM vendor.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyCablivi (caplacizumab-yhdp) — coverage criteria for aTTP

Policy CodePolicy N/A

Change TypeNo material change

Effective DateJul 26, 2024

Next Review DateJul 10, 2024

Key ActionObtain prior authorization; continuation requests may be exempt if medication was used within the last year, no progression or intolerance, and no regimen changes.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

11single-dose max (mg)

30max duration (days)

2allowed courses

requiredcombination therapy

Coverage Criteria for Cablivi (caplacizumab-yhdp)

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aTTP (Initial/Concomitant Therapy)

Covered when ALL of the following are met:

aTTP combination therapy: Member has acquired thrombotic thrombocytopenic purpura (aTTP) and Cablivi is being used in combination with plasma exchange and immunosuppressive therapy

Immunosuppressive therapy examples: systemic corticosteroids, rituximab, cyclosporine, cyclophosphamide, vincristine; provider may taper/discontinue per discretion

Continuation Therapy Exemption

Continuation requests are exempt from Evolent policy when ALL of the following are met:

Continuation exemption: Requested medication was used within the last year

Continuation exemption: Member has not experienced disease progression and/or no intolerance to the requested medication

Continuation exemption: Additional medication(s) are not being added to the continuation request

Cablivi is excluded when the members platelet count is within normal limits or the member is not at risk of bleeding. This policy requires thrombocytopenia or bleeding risk consistent with aTTP to justify therapy; normal platelets or lack of bleeding risk do not meet coverage criteria.

Requests that would result in a single dose greater than 11 mg of Cablivi are excluded. The policy enforces a single-dose maximum limit of 11 mg and doses exceeding this threshold may be denied.

Treatment with Cablivi beyond 30 days after the last plasma exchange is excluded except in a limited circumstance: if ADAMTS13 activity remains suppressed after the last plasma exchange, an additional 28-day extension may be allowed, for a maximum of two treatment courses. Requests exceeding the 30-day post-exchange limit must document persistently low ADAMTS13 to qualify for the permitted extension.

Use of Cablivi for investigational or off-label indications is excluded when the evidence is insufficient or not supported by CMS-recognized compendia or acceptable peer-reviewed literature. Insufficient evidence includes: lack of adequate representation of the patient or treatment regimen in published studies, outcomes that are not clinically meaningful, inappropriate experimental design for the question, reliance solely on uncontrolled case reports, or abstracts without a full peer-reviewed article. Such uses will not meet coverage requirements.

Cablivi is considered not medically necessary for investigational or off-label indications that are not generally accepted by the medical community. The policy defines acceptable supporting evidence as data from CMS-recognized compendia or full, peer-reviewed publications that demonstrate appropriate study design and clinically meaningful benefit; absence of these standards supports a determination of not medically necessary.

Coding and Key Limits

Single dose limit — Cablivi

Single-dose maximum11 mg per single dose of Cablivi (caplacizumab-yhdp)

Applies toAll dosing requests submitted for Cablivi

Consequence of exceedanceRequests exceeding this single-dose limit may be excluded or denied

Treatment duration after last plasma exchange

Standard maximum duration after last plasma exchange30 days

Permitted extensionIf ADAMTS13 activity remains suppressed after the last plasma exchange, therapy may be extended an additional 28 days (for a total of up to 2 treatment courses)

Exceedance consequenceTreatment beyond these limits may be excluded or denied

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Cablivi (caplacizumab-yhdp). Continuation requests may be exempt when the medication was used within the last year, there is no disease progression or intolerance, and no additional medications are being added to the continuation request.

Continuation exemption conditions: used within last 12 months; no disease progression or intolerance; no additional medications added to the request.

Note

Therapy Sequencing for aTTP

Cablivi must be used as adjunctive therapy together with plasma exchange and immunosuppressive therapy for acquired thrombotic thrombocytopenic purpura (aTTP). Immunosuppressive agents may include systemic corticosteroids, rituximab, cyclosporine, cyclophosphamide, or vincristine and may be tapered or discontinued at the provider's discretion.

Must be used in combination with plasma exchange and immunosuppressive therapy for aTTP.

Documentation Required

Required Clinical Documentation

Requests must include documentation that supports the diagnosis of aTTP, the use of Cablivi in combination with plasma exchange and immunosuppressive therapy, and rationale for continued therapy when applicable.

Documentation should include: clinical diagnosis of aTTP, evidence of plasma exchange and concurrent immunosuppressive therapy, prior treatment dates, ADAMTS13 activity levels when available, and justification for continuation requests.

Denial Risk

Dose and Duration Exceedance

Requests that exceed the single-dose limit of 11 mg or that extend treatment beyond the permitted duration may be denied. The standard maximum duration is 30 days beyond the last plasma exchange; if ADAMTS13 activity remains suppressed after the last plasma exchange, an additional 28 days (one extra treatment course) may be considered.

Single dose maximum: 11 mg — doses above this may be denied.
Maximum duration: 30 days beyond last plasma exchange; possible single extension of 28 days if ADAMTS13 activity remains suppressed (total of two treatment courses).

Denial Risk

Insufficient Evidence for Off-Label Use

Off‑label or investigational uses lacking support from FDA labeling, CMS‑recognized compendia, NCCN/ASCO guidelines, or acceptable peer‑reviewed evidence may be denied. Evidence should adequately represent the patient's clinical characteristics and treatment regimen, report clinically meaningful outcomes, and be of appropriate study design; case reports alone are generally insufficient.

Insufficient evidence for off‑label use may result in denial if not supported by CMS compendia, NCCN/ASCO guidelines, or robust peer‑reviewed literature.
Evidence considerations include representativeness of patient population, relevance of concomitant therapies, clinically meaningful outcomes (e.g., ASCO-recommended thresholds), and appropriate study design; anecdotal case reports are usually inadequate.

Clinical Background

Acquired thrombotic thrombocytopenic purpura (aTTP) is an immune-mediated disorder characterized by severe ADAMTS13 deficiency leading to microvascular thrombosis and thrombocytopenia. In aTTP, Cablivi (caplacizumab-yhdp) is used as an adjunct to plasma exchange and immunosuppressive therapy to inhibit von Willebrand factormediated platelet aggregation and reduce formation of microthrombi. This policy limits Cablivi coverage to its role as combination therapy for aTTP and applies utilization controls (prior authorization, dose and duration limits) consistent with the FDA label and available evidence.

Definitions and Acceptable Evidence

aTTP definition — acquired thrombotic thrombocytopenic purpura

DefinitionaTTP = acquired thrombotic thrombocytopenic purpura (the indicated condition for Cablivi in this policy)

ContextCablivi is indicated as adjunctive therapy for aTTP when used with plasma exchange and immunosuppressive therapy

Examples of immunosuppressive therapySystemic corticosteroids, rituximab, cyclosporine, cyclophosphamide, vincristine

Acceptable evidence

Acceptable evidence sourcesFDA approved product labeling, CMS-recognized compendia, NCCN or ASCO clinical guidelines, or peer-reviewed literature meeting CMS Medicare Benefit Policy Manual Chapter 15 standards

Policy requirementUse of Cablivi must be supported by one of the acceptable evidence sources to be approvable by Evolent

Reference guidanceCMS Medicare Benefit Policy Manual Chapter 15 defines standards for acceptable peer-reviewed literature

Initial Therapy Criteria

Initial Therapy for aTTP

Initial coverage requires use in combination with other standard therapies for aTTP.

Combination therapy: Cablivi must be used with plasma exchange and immunosuppressive therapy

Provider discretion allowed for tapering/discontinuing immunosuppressive agents

Continuation Therapy Criteria

Continuation Exemption

Continuation requests may be exempt from re-authorization when all criteria are met.

Prior use: Medication used within the last year

Clinical stability: No disease progression and no intolerance to medication

No regimen change: No additional medications are being added to the continuation request

Step Therapy and Sequencing

Step	Requirement	Notes
1
Cablivi (caplacizumab-yhdp) must be given in combination with plasma exchange and immunosuppressive therapy as part of initial aTTP treatment.
Examples of immunosuppressive therapy include systemic corticosteroids, rituximab, cyclosporine, cyclophosphamide, or vincristine; immunosuppressive therapy may be tapered or discontinued at the physician/provider's discretion.

Quantity Limits

Cablivi — quantity / single unit value

ProductCablivi (caplacizumab-yhdp)

Single unit concentration/value11 mg per single unit/dose

Quantity limit contextListed as the single-dose quantity limit for coverage and utilization management

Site of Care Considerations