Hematology - Adzynma Utilization Management Medical Policy
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Defines prior authorization, coverage criteria, dosing, and documentation requirements for Adzynma for treatment of congenital thrombotic thrombocytopenic purpura for Aspirus Medicare Plans.
No material clinical or coverage changes in this revision.
Coverage and Authorization Criteria
Initial approval — Congenital Thrombotic Thrombocytopenic Purpura
Approve for 1 year if the patient meets ALL of the following (A, B, C, and D):
Documentation for each element is required; baseline refers to pre-treatment values.
Approved dosing
Dosing. Approve the following dosing regimens (A and/or B):
On-demand therapy described as daily dosing until 2 days after event resolves; total weekly dose limit applies.
Coverage of Adzynma is limited to the circumstances explicitly listed in the Recommended Authorization Criteria. Requests for use outside those criteria are not recommended for approval. The criteria will be revised as new published data become available, and providers should anticipate updates that may change covered indications or requirements.
Diagnostic Thresholds and Coding
Provider Requirements, Documentation, and Exceptions
Prior Authorization Required
Prior authorization is recommended. Approval is granted for 1 year when all specified diagnostic and prescribing criteria are met and dosing conforms to the documented regimens. Requests for doses outside the documented dosing will be considered on a case-by-case basis by a clinician (e.g., Medical Director or Pharmacist). Adzynma must be prescribed by or in consultation with a physician who specializes in the condition being treated (hematologist for congenital TTP). Automation: None.
- Approve for 1 year when ALL FDA‑approved indication criteria are met (ADAMTS13 activity < 10%; no anti‑ADAMTS13 autoantibodies; pathogenic/likely pathogenic ADAMTS13 gene variant; prescribed by/with hematologist).
- Dosing approvals: routine prophylaxis up to 40 IU/kg IV once weekly; on‑demand therapy up to 135 IU/kg IV per week (total weekly dose not to exceed 135 IU/kg).
- Requests for doses outside documented dosing considered case‑by‑case by clinician.
Dose Exceptions / Step Therapy
No automated step therapy is specified. Requests that propose a different step or sequencing of therapies than described in this policy will be reviewed individually.
Required Documentation
Documentation is required as noted in the criteria. Acceptable documentation may include chart notes, laboratory data, diagnostic test results, and genetic test results demonstrating baseline ADAMTS13 activity, absence of anti‑ADAMTS13 autoantibodies, and presence of a pathogenic ADAMTS13 gene variant. Documentation must support the diagnosis and baseline measurements prior to therapy.
- Baseline ADAMTS13 activity (< 10%) [documentation required].
- Diagnostic test showing absence of anti‑ADAMTS13 autoantibodies [documentation required].
- Genetic testing showing pathogenic ADAMTS13 variant [documentation required].
- Evidence the medication is prescribed by or in consultation with a hematologist.
Denial Trigger
Requests that do not meet the Recommended Authorization Criteria, or for indications or circumstances not listed in the criteria, are not recommended for approval. Criteria may be updated as new published data become available.
- Coverage is not recommended for circumstances not listed in the Recommended Authorization Criteria.
- Denial likely if documentation does not demonstrate baseline ADAMTS13 < 10%, absence of anti‑ADAMTS13 autoantibodies, or pathogenic ADAMTS13 gene variant, or if prescribing does not involve a hematologist.
Clinical Background
Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare, inherited disorder caused by pathogenic variants in the ADAMTS13 gene that result in severe ADAMTS13 enzyme deficiency (<10% activity). The deficiency leads to microvascular thrombosis with attendant risks of thrombocytopenia, bleeding, stroke, and end-organ damage. Traditional management has relied on plasma-based prophylaxis or on-demand plasma therapy; Adzynma provides recombinant ADAMTS13 replacement for prophylactic or on-demand use.
Key Definitions
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