CurrentCignaPolicy IP0161

Cablivi (caplacizumab-yhdp) coverage for acquired TTP

Defines Cigna's coverage policy and prior authorization criteria for Cablivi (caplacizumab-yhdp) when used to treat acquired thrombotic thrombocytopenic purpura (aTTP) in adults, including dosing limits, prescribing clinician requirements, and coding information.

Policy Summary

PayerCigna

PolicyCablivi (caplacizumab-yhdp) coverage for acquired TTP

Policy CodePolicy IP0161

Change TypeAnnual Revision (no criteria changes)

Effective DateMay 15, 2025

Next Review Date

Key ActionPrior Authorization is recommended; ensure prescribing by or consultation with a hematologist and document inpatient initiation with plasma exchange and current immunosuppressive therapy.

POLICY UPDATE CHANGES

Annual Revision, Summary of Changes = No criteria changes.

1Covered Indication (aTTP)

18+Age requirement (years)

60Max doses after last plasma exchange

28Max extension days if suppressed ADAMTS13

Coverage Summary

This policy sets Cigna's coverage position for Cablivi (caplacizumab-yhdp) as covered with criteria for the FDA‑approved indication of treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in adults, in combination with plasma exchange and immunosuppressive therapy. Approvals require that the patient is ≥ 18 years of age, Cablivi was initiated inpatient in combination with plasma exchange, the patient is receiving at least one immunosuppressive therapy, the medication is prescribed by or in consultation with a hematologist, and prior receipt of Cablivi did not include more than two recurrences while on therapy. The policy defines prior authorization requirements, dosing limits and coding (HCPCS C9047) for coverage determinations.

Initial (FDA-Approved) Therapy Criteria

FDA-Approved Indication - Approve for one course of treatment (up to 60 days following the last plasma exchange session)

Approve if ALL of the following are met (A, B, C, D, and E):

A: Age requirement: Patient ≥ 18 years of age>= 18 years
B: Inpatient initiation and plasma exchange: Cablivi was initiated in the inpatient setting, in combination with plasma exchange therapy
C: Immunosuppressive therapy: Patient is currently receiving at least one immunosuppressive therapy
Examples include systemic corticosteroids, rituximab (or a rituximab product), cyclosporine, cyclophosphamide, mycophenolate mofetil, hydroxychloroquine, bortezomib.
D: Prior Cablivi recurrences: If the patient has previously received Cablivi, he/she has not had more than two recurrences of acquired thrombotic thrombocytopenic purpura while on Cablivi<= 2 recurrences while on Cablivi
E: Prescribing clinician: The medication is prescribed by or in consultation with a hematologist

Prior Authorization

Prior authorization recommended

Prior Authorization is recommended for prescription benefit coverage of Cablivi; approvals are provided for the duration noted and require prescribing by or consultation with a hematologist.

C9047

Dosing, Extension & Discontinuation Rules

Dosing - Approve the following dosing regimens

ALL of the following dosing rules apply when approving:

Day 1 two-dose rule: Day 1 of treatment with plasma exchange: Two doses of Cablivi (11 mg IV bolus prior to plasma exchange followed by an 11 mg SC dose after completion of plasma exchange)
Daily dosing: 11 mg SC injection up to once dailyup to once daily
Maximum doses after last plasma exchange: Do not exceed 60 doses following the last plasma exchange sessionDo not exceed 60 doses

Extension / Discontinuation

Additional rules regarding duration:

Continue after plasma exchange: Treatment is continued for 30 days after the last plasma exchange session30 days
Extension if suppressed ADAMTS13: If, after the initial treatment course, there are signs of persistent underlying disease such as suppressed ADAMTS13 levels, Cablivi therapy may be extended for a maximum of 28 daysup to 28 days
Discontinue after multiple recurrences: Cablivi should be discontinued if the patient experiences more than two recurrences of aTTP while on Cablivi> 2 recurrences while on Cablivi

Billing Rule

Dosing/billing limit

Do not exceed 60 doses following the last plasma exchange session when authorizing/providing therapy.

Do not exceed 60 doses following the last plasma exchange session
C9047

Unproven / Not Medically Necessary Uses

Not Medically Necessary

Use not meeting the above criteria:

Not Medically Necessary: Cablivi for any other use is considered not medically necessary

Denial Risk

Not covered if outside indication

Claims for Cablivi will be denied as not covered when billed for conditions or diagnoses that are not covered under this Coverage Policy.

Provider Requirements & Documentation

Documentation Required

Documentation of immunosuppressive therapy

Provider must document that the patient is currently receiving at least one immunosuppressive therapy (examples listed in policy).

Systemic corticosteroids
Rituximab (or a rituximab product)
Cyclosporine
Cyclophosphamide
Mycophenolate mofetil
Hydroxychloroquine
Bortezomib

Documentation Required

Inpatient initiation and plasma exchange

Provider must document Cablivi was initiated in the inpatient setting in combination with plasma exchange therapy.

Note

Clinical Evidence & Guidance

Evidence sources referenced in the policy:

• FDA Prescribing Information — Cablivi prescribing information, Genzyme/Sanofi; April 2024 (prescribing information cited in policy).

• ISTH guideline — International Society on Thrombosis and Haemostasis recommendation: the 2020 ISTH guideline suggests using Cablivi for acute aTTP events (the guideline panel recommends Cablivi for patients experiencing an acute event, with administration under guidance of experienced clinicians).

Background

Background: Cablivi is a von Willebrand factor (vWF)-directed antibody fragment (a nanobody) that targets ultra‑large vWF multimers to inhibit vWF–platelet interactions. It is indicated for treatment of acquired TTP in adults in combination with plasma exchange and immunosuppressive therapy. Standard aTTP care includes plasma exchange and glucocorticoids, with rituximab often added as an immunosuppressant. Dosing follows a pattern of two doses on day 1 of plasma exchange (an 11 mg IV bolus prior to plasma exchange followed by an 11 mg subcutaneous dose after completion of plasma exchange), then 11 mg SC up to once daily during plasma exchange and continued for 30 days after the last plasma exchange session, with possible extension up to 28 days if suppressed ADAMTS13 levels persist (and discontinuation if more than two recurrences occur while on Cablivi).

Definitions

aTTPAcquired thrombotic thrombocytopenic purpura (immune-mediated TTP due to autoantibodies inhibiting ADAMTS13).

Revision History

2024-06-15Annual Revision

Annual Revision noted on 06/15/2024: Annual Revision, Summary of Changes = No criteria changes.

2025-05-15Annual RevisionLatest

Annual Revision noted on 05/15/2025: Annual Revision, Summary of Changes = No criteria changes.