Adzynma (ADAMTS13, recombinant-krhn) — Medical prior authorization and coverage criteria for congenital TTP
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Defines medical prior authorization and coverage criteria for Adzynma used as prophylactic or on‑demand enzyme replacement therapy in adult and pediatric patients with congenital thrombotic thrombocytopenic purpura (cTTP) for CareSource Arkansas PASSE.
No material clinical or coverage changes in this revision.
Coverage Criteria for Adzynma (ADAMTS13, recombinant-krhn)
Initial Therapy
Covered when ALL of the following are met for initial authorization:
Continuation Therapy / Reauthorization
Reauthorization covered when ALL of the following are met:
If met, approve for additional 12 months.
Coverage of Adzynma (ADAMTS13, recombinant-krhn) is provided when the documented clinical presentation and supporting tests satisfy the medical necessity criteria in this policy. Specifically, initial and continuation authorizations require documentation that the medication is being used to treat congenital thrombotic thrombocytopenic purpura (cTTP) and that prescriber and diagnostic requirements in the criteria are met. Off‑label uses (indications not listed in this policy) must be evaluated under the plan’s Off‑Label policy and do not meet the medical necessity criteria described here unless explicitly approved through that process.
Use of Adzynma for indications other than congenital TTP as described in this document is considered not medically necessary. Requests for other diagnoses should be referred to the Off‑Label policy and will not be approved under the standard coverage criteria herein.
Initial Therapy Requirements and Dosing
Initial Therapy
Initial dosing and prescriber requirements
Initial approval duration: 6 months.
Reauthorization and Ongoing Use
Reauthorization
Reauthorization requirements for ongoing coverage
Approval for 12 months if criteria met.
Provider Requirements and Administrative Actions
Medical prior authorization required
Adzynma (ADAMTS13, recombinant-krhn) requires medical prior authorization under the medical benefit for treatment of congenital TTP when policy criteria are met.
No step therapy required
This policy does not impose any step therapy requirements for Adzynma.
Required documentation for initial request and reauthorization
Initial authorization requests must include documentation that the medication was prescribed by or in consultation with a hematologist, genetic test results demonstrating an ADAMTS13 gene mutation, and a plasma ADAMTS13 activity level <10% of normal (<10 IU/dL). Reauthorization requests must include chart notes showing clinical improvement or stabilization (fewer acute TTP events, increased ADAMTS13 activity, or increased platelet count).
- Prescriber: hematologist or consultation documentation
- Genetic test results confirming ADAMTS13 gene mutation
- Plasma ADAMTS13 activity level <10% of normal (<10 IU/dL) for initial requests
- Reauthorization: chart notes demonstrating improvement or stability (fewer acute TTP events, increased ADAMTS13 activity, or increased platelet count)
Denial triggers for Adzynma authorization
Authorization may be denied if required documentation or coverage criteria are not provided — for example, lack of hematologist involvement, missing genetic confirmation of ADAMTS13 mutation, or plasma ADAMTS13 activity ≥10% of normal; similarly, failure to supply reauthorization chart notes showing improvement/stability can lead to denial.
- No documentation of hematologist prescription or consultation
- No genetic test demonstrating ADAMTS13 mutation
- Plasma ADAMTS13 activity not shown to be <10% of normal
- Missing reauthorization chart notes showing clinical improvement or stability
Diagnostic and Laboratory Coding / Thresholds
Administration Setting
IV administration — no site restriction specified
Adzynma is administered intravenously; the policy specifies IV administration but does not further restrict the site of care (no additional infusion center/site limitations stated).
Key Definitions
Quantity and Dosing Limits
Step Therapy Requirements
| Step | Requirement |
|---|---|
| No step therapy steps specified | This policy does not include any step therapy requirements for Adzynma; medical prior authorization and coverage criteria apply as outlined elsewhere in the policy. |
Background and Clinical Context
Adzynma (ADAMTS13, recombinant-krhn) is a recombinant ADAMTS13 product indicated in this policy for enzyme replacement in patients with congenital thrombotic thrombocytopenic purpura (cTTP). cTTP is a hereditary deficiency of ADAMTS13 that results in accumulation of ultra‑large von Willebrand factor multimers, platelet aggregation, microthrombi formation, hemolytic anemia, thrombocytopenia and potential organ dysfunction. Historically, plasma infusions were used to replace ADAMTS13; Adzynma provides a targeted recombinant enzyme replacement option for prophylactic or on‑demand treatment in this population.
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