Cablivi (caplacizumab-yhdp)

POLICY NUMBER

L.5.01.543

Cablivi (caplacizumab-yhdp)

Please perform a formulary drug search on your patient’s member ID to ensure the prescription drug is covered under their benefit plan. The medication(s) in this medical policy may not be covered under a specific member’s benefit plan.

DESCRIPTION

Cablivi (caplacizumab-yhdp) is a von Willebrand factor (vWF)-directed antibody fragment indicated in combination with plasma exchange and immunosuppressive therapy for the treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP).

Acquired thrombotic thrombocytopenic purpura (aTTP) is a blood disorder in which the body creates antibodies to block the ADAMTS13 enzyme, which helps control clotting factors. Characteristics of aTTP include reduced platelet count, bleeding under the skin, and hemolytic anemia. Cablivi (caplacizumab-yhdp) targets the vWF clotting factor to inhibit the interaction between vWF and platelets, resulting in decreased platelet consumption.

The initial dose of Cablivi (caplacizumab-yhdp) must be administered as a bolus intravenous injection by a healthcare professional. Subsequent doses can be administered subcutaneously.

POLICY

Prior authorization is required.

The use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. 

Initial Request

Cablivi (caplacizumab-yhdp) may be considered medically necessary when ALL of the following criteria are met:

1. The individual is 18 years of age or older;

2. The individual has a diagnosis (or suspected diagnosis) of acquired thrombotic thrombocytopenic purpura (aTTP) as documented by ALL of the following:

   1. Individual presents with severe thrombocytopenia (platelet count <100 x 109/L);

   2. Individual presents with microangiopathic hemolytic anemia (MAHA) confirmed by red blood cell fragmentation (e.g. schistocytes) on peripheral blood smear; AND

   3. Testing for ADAMTS13 activity levels has been completed or is in progress;

3. BOTH of the following:

   1. The individual will be receiving the requested agent in combination with plasma exchange and immunosuppressive therapy (e.g. corticosteroids, rituximab, etc.); AND

   2. The individual will not receive the requested agent beyond 30-days following the last daily plasma exchange;

4. If the individual has previously received Cablivi (caplacizumab-yhdp), the individual has not had >2 recurrences or exacerbations [defined as thrombocytopenia after initial recovery of platelet count (platelet count ≥150,000/µL) that requires reinitiation of daily plasma exchange] of aTTP while on therapy with Cablivi (caplacizumab-yhdp) [a 28-day extension of therapy does not count as a recurrence];

5. The prescriber is a specialist or has consulted with a specialist in area of the individual's diagnosis (e.g., hematologist);

6. The individual does not have any contraindication(s) to therapy with the requested agent; AND

7. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: Up to 30 days from last plasma exchange.

Renewal Evaluation

Cablivi (caplacizumab-yhdp) may be approved for renewal when ALL of the following criteria are met:

1. The individual has been previously approved for the requested agent through the BCBSMS PA process;

2. The individual continues to have signs of persistent underlying disease (i.e., suppressed ADAMTS13 activity levels);

3. The individual has not had >2 recurrences/exacerbations of aTTP while on the requested agent (defined as thrombocytopenia after initial recovery of platelet count [platelet count ≥150,000/µL] that requires reinitiation of daily plasma exchange);

4. The prescriber is a specialist or has consulted with a specialist in area of the individual’s diagnosis (e.g., hematologist);

5. The individual does not have any contraindication(s) to therapy with the requested agent; AND

6. The prescribed dosage is within the program quantity limits based on FDA approved labeled dosage.

Length of Approval: One time renewal of up to 28 days.

POLICY EXCEPTIONS

State Health Plan (State and School Employees) Participants

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

1. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

2. appropriate with regard to standards of good medical practice; and

3. not solely for the convenience of the Member, his or her Provider; and

4. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient. 

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary. BCBSMS may request medical records for determination of medical necessity. When medical records are requested, letters of support and/or explanation are often useful, but are not sufficient documentation unless all specific information needed to make a medical necessity determination is included.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [ie, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

03/02/2020: New policy added.

05/18/2020: Policy description updated regarding acquired thrombotic thrombocytopenic purpura. Policy section updated to add renewal evaluation criteria. Policy Exceptions updated regarding State Health Plan members. Sources updated.

05/15/2021: Policy reviewed and approved by Pharmacy & Therapeutics (P&T) Committee. Policy description updated to include generic name for Cablivi. Policy section updated to revise criteria for initial requests and renewal evaluation. For renewal evaluations, the length of approval is a one time renewal of up to 28 days. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and to add information regarding BCBSMS request for medical records. Sources updated. Code Reference section updated to add HCPCS code C9047.

02/01/2023: Policy Exceptions updated to add the following: State Health Plan (State and School Employees): The prescription drug(s) in this medical policy may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary. Services related to delivery and/or administration of a self-administered drug are not covered.

07/01/2023: Policy Exceptions updated regarding State Health Plan (State and School Employees) Participants.

06/03/2025: Policy reviewed and approved by the Pharmacy & Therapeutics (P&T) Committee. Policy description updated regarding administration of Cablivi (caplacizumab-yhdp). Policy statement revised to state that the use of samples by an individual will not be considered current or stable therapy to satisfy Medical Policy requirements. Medically necessary criteria updated regarding documentation of diagnosis (or suspected diagnosis) of acquired thrombotic thrombocytopenic purpura (aTTP). Re-worded policy criteria regarding use of the requested agent in combination with plasma exchange and immunosuppressive therapy. Revised statement regarding individuals with previous use of Cablivi (caplacizumab-yhdp). Renewal criteria updated regarding recurrences/exacerbations of aTTP while on the requested agent, prescriber requirements, contraindications to therapy, and dose requirements. Sources updated.

SOURCE(S)

1. Acquired thrombotic thrombocytopenic purpura. Genetic and Rare Disease Information Center. National Center for Advancing Translational Sciences. Last accessed March 2025. https://rarediseases.info.nih.gov/diseases/4607/thrombotic-thrombocytopenic-purpura-acquired .

2. Cablivi prescribing information. Genzyme Corporation. November 2024. Last accessed March 2025.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

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