CurrentAnthemPolicy CC-0137

Medical Drug Clinical Criteria - Cablivi (caplacizumab-yhdp)

Clinical criteria for medical-benefit coverage and prior authorization of Cablivi (caplacizumab-yhdp) for treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP), including initial and continuation therapy requirements, dosing context (with plasma exchange and immunosuppression), and relevant billing codes.

Policy Summary

PayerAnthem

PolicyMedical Drug Clinical Criteria - Cablivi (caplacizumab-yhdp)

Policy CodePolicy CC-0137

Change Typeclarified (coding updates)

Effective DateJul 1, 2025

Next Review Date

Key ActionRequests will be reviewed for medical necessity against the specified clinical criteria (age ≥18, diagnosis or suspected aTTP, lab and smear documentation, ADAMTS13 testing status, combination with plasma exchange and immunosuppression or post-plasma-exchange 30-day use with ≤2 recurrences).

POLICY UPDATE CHANGES

05/16/2025 - Annual Review: no change. Coding Reviewed: Updated descriptions of HCPCS C9047 and J3590. Added ICD-10-CM D69.3.

1Covered Indication (aTTP)

18+Age requirement (years)

≤2Allowed recurrences while on therapy

28Max additional continuation days

Scope: Coverage criteria address prior authorization for Cablivi (caplacizumab-yhdp) for the treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP), including initial and continuation therapy requirements, dosing context with plasma exchange and immunosuppressive therapy, and relevant billing codes.

Coverage stance: covered_with_criteria for adults (age ≥18) with a diagnosis or suspected aTTP when used in combination with plasma exchange and immunosuppressive therapy during the plasma exchange period, and for up to 30 days after completion of plasma exchange (with possible extension up to 28 additional consecutive days when aTTP is unresolved).

Required treatment context: Cablivi must be used in combination with plasma exchange and immunosuppressive therapy (during daily plasma exchange period) or used after plasma exchange for 30 days post-exchange with limits on recurrences/exacerbations as specified.

Dosing context (high-level): Administer as an 11 mg IV injection on day 1 prior to plasma exchange, followed by an 11 mg SC injection after that day's plasma exchange; on subsequent plasma exchange days give 11 mg SC daily following exchange; continue 11 mg SC daily for 30 days after plasma exchange completion and may extend weekly up to 28 additional days if aTTP remains unresolved.

Key thresholds

Age requirement≥ 18 years

Severe thrombocytopenia thresholdPlatelet count <100 x 10^9/L

Recurrence/exacerbation definitionThrombocytopenia after initial recovery of platelet count (≥150,000) requiring re-initiation of daily plasma exchange

Maximum continuation extensionMaximum 28 additional consecutive days

Initial Therapy Criteria

Initial Authorization (Requests for Cablivi may be approved if)

Requests for Cablivi (caplacizumab-yhdp) may be approved if the following criteria are met:

ALL of the following

Individual is 18 years of age≥ 18 years
Individual has a diagnosis or suspected diagnosis of acquired thrombotic thrombocytopenic purpura (aTTP)
ALL of the following
- Documentation is provided that individual presents with severe thrombocytopenia (platelet count <100 x 10^9/L) (ISTH 2020)platelet count <100 x 10^9/L
- Documentation is provided that individual presents with microangiopathic hemolytic anemia (MAHA) shown by red blood cell fragmentation (e.g. schistocytes) on peripheral blood smear
- Individual is testing for ADAMTS13 activity levels has been completed or in progress
AND (one of the following treatment contexts must apply)
- Individual is using in combination with plasma exchange and immunosuppressive therapy for the duration of the daily plasma exchange period
- Individual is using after completion of plasma exchange for 30 days and has not had more than 2 recurrences/exacerbations of aTTP while on Cablivi therapy (recurrence/exacerbation defined as thrombocytopenia after initial recovery of platelet count (platelet count ≥ 150,000) that requires re-initiation of daily plasma exchange)≤ 2 recurrences/exacerbations while on therapy

Continuation Therapy Criteria

Continuation Authorization (Requests for continuation of subcutaneous Cablivi may be approved if)

Requests for continuation of Cablivi (caplacizumab-yhdp) subcutaneous use may be approved if the following criteria are met:

ALL of the following

Individual has received Cablivi initial treatment course (in combination with plasma exchange/immunosuppressive therapy, and for 30 days beyond the last plasma exchange)
Documentation is provided that individual has signs of persistent underlying disease (e.g. ongoing suppressed ADAMTS13 activity levels) present after initial treatment course
Documentation is provided that individual has not had more than 2 recurrences/exacerbations of aTTP while on caplacizumab-yhdp therapy (recurrence/exacerbation defined as thrombocytopenia after initial recovery of platelet count (platelet count ≥ 150,000) that requires re-initiation of daily plasma exchange)≤ 2 recurrences/exacerbations while on therapy
Individual is using for a maximum of 28 total additional days (given consecutively)maximum 28 additional consecutive days

Not Approved / Exclusions

Not Approved

Cablivi may not be approved when the above criteria are not met and for all other indications.

Any request not meeting the specified initial or continuation criteria

Policy covers only the specified indications and contexts

Provider Actions

Prior Authorization

Prior authorization required under medical-benefit review

Requests will be reviewed for medical necessity against the specified clinical criteria (age ≥18, diagnosis or suspected aTTP, lab and smear documentation, ADAMTS13 testing status, combination with plasma exchange and immunosuppression or post-plasma-exchange 30-day use with ≤2 recurrences).

C9047
J3590

Documentation Required

Required clinical documentation

Provide platelet counts demonstrating platelet count <100 x 10^9/L, peripheral blood smear showing microangiopathic hemolytic anemia (MAHA) with schistocytes, documentation that ADAMTS13 activity testing has been completed or is in progress, and documentation of plasma exchange and immunosuppressive therapy use or the treatment timeline and recurrence history.

Platelet counts showing platelet count <100 x 10^9/L
Peripheral blood smear showing MAHA (schistocytes)
Documentation that ADAMTS13 activity testing is completed or in progress
Documentation of plasma exchange and immunosuppressive therapy use, treatment timeline, and recurrence history

Billing Rule

Use of HCPCS/J-code

Report HCPCS code C9047 (Injection, caplacizumab-yhdp, 1mg [Cablivi]) or J3590 (unclassified biologics) when specifying Cablivi as the product per coding guidance.

Report C9047 or J3590 when specifying Cablivi as the product

Denial Risk

Denial if criteria unmet

Claims may be denied if initial or continuation clinical criteria are not met, including age <18, lack of documentation of severe thrombocytopenia (platelet count <100 x 10^9/L) or MAHA, absence of ADAMTS13 testing, not used in combination with required plasma exchange and immunosuppression (or appropriate post-plasma-exchange use), or exceeding allowed recurrences/extension limits.

Age <18 years
Missing documentation of platelet count <100 x 10^9/L
Missing peripheral smear showing MAHA (schistocytes)
No documentation of ADAMTS13 testing (completed or in progress)
Not used with required plasma exchange and immunosuppressive therapy or not within the 30-day post-plasma-exchange context
More than 2 recurrences/exacerbations while on therapy or exceeding the maximum 28 additional consecutive days for continuation

Clinical Evidence

Evidence summary: The policy references the International Society on Thrombosis and Haemostasis (ISTH) 2020 guideline for defining the thrombocytopenia threshold used in the clinical criteria (platelet count <100 x 10^9/L).

The ISTH 2020 guidance is cited in the document references and informed the platelet threshold selected for determining severe thrombocytopenia in the approval criteria.

Additional supporting references for Cablivi dosing and indication include standard drug information sources (Clinical Pharmacology, DailyMed) listed in the references section.

Definitions & Background

Background: Cablivi (caplacizumab-yhdp) is a vWF-directed antibody fragment indicated by the FDA for treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP) in combination with plasma exchange and immunosuppressive therapy. It targets the A1-domain of vWF to inhibit vWF–platelet interaction and reduce platelet adhesion and consumption.

Dosing schedule: On day 1 administer 11 mg IV prior to plasma exchange and an 11 mg SC injection after that day's plasma exchange. On subsequent plasma exchange days give 11 mg SC daily following plasma exchange. After completion of plasma exchange continue 11 mg SC daily for 30 days; if aTTP is not resolved the treatment may be extended weekly for a maximum of 4 weeks (28 days continuously) in combination with other immunosuppressive therapy.

Definitions: Severe ADAMTS13 deficiency = ADAMTS13 activity <10%, which typically confirms the diagnosis of TTP in the appropriate clinical setting; MAHA (microangiopathic hemolytic anemia) = non-immune hemolysis with red blood cell fragmentation producing schistocytes on peripheral blood smear; Recurrence/exacerbation (aTTP) = thrombocytopenia after initial recovery of platelet count (platelet count ≥150,000) that requires re-initiation of daily plasma exchange.