Overview

This document addresses the use of Cablivi (caplacizumab-yhdp). Cablivi is an injectable (intravenous (IV) and subcutaneous (SC)) von Willebrand factor (vWF)-directed antibody fragment. Cablivi is FDA indicated for the treatment of adults with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy. Caplacizumab-yhdp targets the A1-domain of von Willebrand factor (vWF) and inhibits the interaction between vWF and platelets, thus reducing both vWF-mediated platelet adhesion and platelet consumption. In aTTP, autoantibodies inhibit activity of the vWF-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), which leads to unrestrained adhesion of vWF to platelets and microvascular thrombosis (increased risk of blood clots). Caplacizumab-yhdp is administered on the first day of treatment by a healthcare professional as an 11 mg IV injection prior to plasma exchange followed by an 11 mg SC injection after completion of plasma exchange on that day. On subsequent treatment days during plasma exchange caplacizumab is administered as an 11 mg SC injection following daily plasma exchange. Additional caplacizumab-yhdp treatment is administered as a daily 11 mg SC injection for 30 days after the plasma exchange period has been completed. If at the end of the treatment period aTTP was not resolved, treatment could be extended weekly for a maximum of 4 weeks (28 days continuously) and administered in combination with other immunosuppressive therapy.

Definitions and Measures

ADAMTS13 activity:

ADAMTS13 assays report the activity of the protease as a percentage of normal, based on unaffected individuals. The following test interpretations are often used:

• Severe deficiency (activity <10 percent) – This finding typically confirms the diagnosis of TTP in the appropriate clinical setting (MAHA and thrombocytopenia without another obvious cause) and the appropriateness of therapy with plasma exchange and immunosuppressive therapy (e.g., glucocorticoids and rituximab).
• Low activity (between 10 and 60 percent) – This finding is seen in many hospitalized patients with inflammatory disorders (eg, sepsis, malignancy).
• Normal (>60 percent) – This finding suggests an etiology for the clinical findings other than TTP, with very rare exceptions

Acquired Thrombotic Thrombocytopenic Purpura (aTTP):

Acquired TTP is the thrombotic microangiopathy caused by autoantibodies to the von Willebrand factor protease ADAMTS13.

Microangiopathic hemolytic anemia (MAHA):

MAHA is a descriptive term for non-immune hemolysis resulting from intravascular red blood cell fragmentation that produces schistocytes on the peripheral blood smear.

Clinical Criteria

When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to clinical review (including prior authorization), the following criteria will be used to determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose.

Cablivi (caplacizumab-yhdp)

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

1. Individual is 18 years of age; AND
2. Individual has a diagnosis or suspected diagnosis of acquired thrombotic thrombocytopenic purpura (aTTP), confirmed by the following:
• Documentation is provided that individual presents with severe thrombocytopenia (platelet count <100 x 109/L) (ISTH 2020); AND

2. Documentation is provided that individual presents with microangiopathic hemolytic anemia (MAHA) confirmed by red blood cell fragmentation (e.g., schistocytes) on peripheral blood smear; AND
3. Individual's testing for ADAMTS13 activity levels has been completed or in progress;

C.

AND

III. IV.

Individual is using in combination with plasma exchange and immunosuppressive therapy for the duration of the daily plasma exchange period;
OR
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 is defined as thrombocytopenia after initial recovery of platelet count (platelet count ">= 150,000) that requires re-initiation of daily plasma exchange).

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

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

Cablivi (caplacizumab-yhdp) may not be approved when the above criteria are not met and for all other indications.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

HCPCS

• J3590
• C9047

Unclassified biologics [when specified as Cablivi]
Injection, caplacizumab-yhdp, 1mg

ICD-10 Diagnosis

• M31.10-M31.19

All Diagnosis for NOC code only
Thrombotic microangiopathy, unspecified

Document History

Revised: 05/19/2023