State and School Employees' Health Insurance Plan - Cablivi (caplacizumab-yhdp)

POLICY NUMBER

S.5.01.543

Cablivi (caplacizumab-yhdp)

Please perform a search of the State Health Plan Medical Drug Formulary for drugs administered and billed through the medical setting.

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

POLICY

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

Initial Criteria

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

   2. The 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 review 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. 

Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary.

POLICY EXCEPTIONS

None

POLICY GUIDELINES

Medical Policy Manual coverage guidelines should not be used in lieu of the Participant's specific benefit plan language outlined in the Mississippi's State and School Employees’ Life and Health Insurance Plan.

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 Participant'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:

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

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

C. not solely for the convenience of the Participant, his or her Provider; and

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

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 [i.e, 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

07/01/2023: New policy added.

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

CPT copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.