ModifiedAnthemPolicy CC-0137

Cablivi (caplacizumab-yhdp)

Clinical medical benefit policy for use of Cablivi (caplacizumab-yhdp) for treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in patients ≥12 years, including initial and continuation criteria, coding references, and exclusions.

Policy Summary

PayerAnthem

PolicyCablivi (caplacizumab-yhdp)

Policy CodePolicy CC-0137

Change TypeAge update to ≥12

Effective Date

Next Review Date

Key ActionProvide documentation of age (≥12), diagnosis or suspected aTTP, platelet count (<100 x 10^9 /L), peripheral smear showing MAHA (schistocytes), ADAMTS13 activity results or that testing is in progress, and planned concomitant plasma exchange and immunosuppressive therapy.

SourceLink

POLICY UPDATE CHANGES

Updated age per label from 18 and older to 12 and older (02/20/2026).

05/16/2025 coding: Updated descriptions of HCPCS C9047 and J3590 and added ICD-10-CM D69.3.

1Indication covered (aTTP)

12Minimum age (years)

2Max recurrences allowed while on therapy

28Max additional consecutive days for continuation

Coverage Summary

This policy covers Cablivi (caplacizumab-yhdp) for treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in patients aged ≥12 years when used in combination with plasma exchange and immunosuppressive therapy and when the specified clinical criteria are met; FDA indication is for adults and pediatric patients ≥12 with aTTP in combination with plasma exchange and immunosuppressive therapy.

Quick facts and thresholds

Minimum age12 years

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

Max recurrences allowed while on therapy2 recurrences/exacerbations

Max additional consecutive days for continuationMaximum of 28 additional consecutive days

Initial Therapy Criteria

Initial Approval (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 12 years of age and older
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 timing conditions)
- Individual is using in combination with plasma exchange and immunosuppressive therapy for the duration of the daily plasma exchange period
- OR: post-plasma exchange use
  - Individual is using after completion of plasma exchange for 30 days30 days
  - Individual 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

Prior Authorization

Clinical documentation for initial approval

Submit documentation of age (≥12), diagnosis or suspected aTTP, platelet count (<100 x 10^9 /L), peripheral smear showing MAHA (schistocytes), and ADAMTS13 activity testing results or evidence testing is in progress; document planned concomitant plasma exchange and immunosuppressive therapy or post-plasma exchange plan.

Age ≥12
Diagnosis or suspected aTTP
Platelet count <100 x 10^9 /L
Peripheral smear showing MAHA (schistocytes)
ADAMTS13 activity results or testing in progress
Planned concomitant plasma exchange and immunosuppressive therapy or post-plasma exchange plan

Continuation Therapy Criteria

Continuation Approval (Requests for continuation of Cablivi subcutaneous use 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
AND
- Individual is using for a maximum of 28 total additional days (given consecutively)maximum of 28 total additional days (consecutive)

Prior Authorization

Continuation documentation

For extension beyond the initial 30 days post-plasma exchange, provide documentation of prior initial treatment course, evidence of persistent suppressed ADAMTS13 activity or other signs of ongoing disease, and documentation that the member has had ≤2 recurrences while on therapy; limit extension to 28 additional consecutive days.

Evidence of completed initial treatment course (plasma exchange + immunosuppressive therapy and 30 days post-plasma exchange)
Documented persistent suppressed ADAMTS13 activity or other signs of ongoing disease
Documentation of ≤2 recurrences/exacerbations while on therapy
Maximum extension: 28 additional consecutive days

Denial Risk

Denial if criteria unmet

Claims or requests will be denied if policy criteria are not met. Specific denial triggers include: age below 12 years, absence of a diagnosis or suspected aTTP, failure to document required laboratory findings (platelet count threshold or peripheral smear evidence of MAHA, and ADAMTS13 testing/result not completed or in progress), lack of concomitant plasma exchange and immunosuppressive therapy when required, exceeding allowed recurrences (more than 2 while on therapy), or exceeding the maximum extension days (more than 28 additional consecutive days).

Provider Actions & Authorization Requirements

Prior Authorization

Clinical documentation for initial approval

Required supporting documents for initial approval and for continuation/extensions: provide documentation of age (≥12), diagnosis or suspected aTTP, platelet count (<100 x 10^9 /L), peripheral smear demonstrating MAHA (schistocytes), ADAMTS13 activity testing results or evidence testing is in progress, documentation of planned or ongoing concomitant plasma exchange and immunosuppressive therapy (or post-plasma exchange plan), and when requesting extension, documentation of the initial treatment course and evidence supporting persistent disease activity and recurrence status.

Age ≥12
Diagnosis or suspected aTTP
Platelet count <100 x 10^9 /L
Peripheral smear showing MAHA (schistocytes)
ADAMTS13 activity results or testing in progress
Planned concomitant plasma exchange and immunosuppressive therapy or post-plasma exchange plan
For extensions: documentation of initial treatment course, evidence of persistent suppressed ADAMTS13 or ongoing disease, and documentation of ≤2 recurrences while on therapy; limit extension to 28 consecutive days

Denial Risk

Denial if criteria unmet

Denial will be issued if the policy criteria are not met, including use for indications other than aTTP, failure to meet age, diagnostic, laboratory, concomitant therapy, recurrence, or maximum extension criteria (for example: age <12, missing platelet count <100 x 10^9 /L or absence of schistocytes, ADAMTS13 testing not completed or in progress, lack of plasma exchange/immunosuppressive therapy when required, >2 recurrences while on therapy, or extension beyond 28 consecutive days).

Clinical Evidence & Indication

The FDA indicates Cablivi for adults and pediatric patients ≥12 years with acquired TTP to be used in combination with plasma exchange and immunosuppressive therapy. Per the label, initial administration includes an 11 mg IV dose on day 1 prior to plasma exchange followed by an 11 mg SC dose after plasma exchange on day 1, with subsequent daily 11 mg SC doses after each plasma exchange during the plasma exchange period, and then daily 11 mg SC for 30 days after completion of plasma exchange with a possible extension up to a maximum of 28 additional consecutive days if aTTP is not resolved.

Definitions

ADAMTS13 activity: assay reports protease activity as a percentage of normal; severe deficiency is <10%, low is 10–60%, and normal is >60%.

aTTP: acquired thrombotic thrombocytopenic purpura is a thrombotic microangiopathy caused by autoantibodies to ADAMTS13.

MAHA: microangiopathic hemolytic anemia is non-immune hemolysis with intravascular red blood cell fragmentation producing schistocytes on the peripheral blood smear.

Policy Summary

PayerAnthem

PolicyCablivi (caplacizumab-yhdp)

Policy CodePolicy CC-0137

Change TypeAge update to ≥12

Effective Date

Next Review Date

SourceLink

On This Page

D69.3	Immune thrombocytopenic purpura
M31.10-M31.19	Thrombotic microangiopathy, unspecified (range included)

OpenPayer

Cablivi (caplacizumab-yhdp)

Coverage Summary

Initial Therapy Criteria

Continuation Therapy Criteria

Not Approved / Exclusions

Applicable Codes

Provider Actions & Authorization Requirements

Clinical Evidence & Indication

Definitions

Revision History