Adzynma (ADAMTS13, recombinant-krhn) prior authorization
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Defines prior authorization, coverage criteria, dosing, and renewal rules for Adzynma for prophylactic or on‑demand enzyme replacement therapy in pediatric and adult patients with congenital thrombotic thrombocytopenic purpura (cTTP). Applies to prior authorization requests submitted to the payer.
No material clinical or coverage changes in this revision.
Coverage Criteria
Initial Therapy — Covered when ALL of the following are met:
Covered when ALL of the following are met:
All conditions required.
Prophylactic Therapy — For prophylactic use, ALL of the following are required in addition to initial criteria:
For prophylactic use, ALL of the following are required in addition to initial criteria:
Prophylaxis-specific criteria.
On-Demand Therapy — For on‑demand use, ALL of the following are required in addition to initial criteria:
For on‑demand use, ALL of the following are required in addition to initial criteria:
On‑demand event documentation required.
Reauthorization Criteria — Reauthorization/renewal is supported when ANY of the following (per modality) demonstrate clinical benefit/stability:
Reauthorization/renewal is supported when ANY of the following (per modality) demonstrate clinical benefit/stability:
General renewal from prescriber.
Prophylaxis renewal criteria include reduction of events.
On‑demand renewal criteria.
Renewal from Previous Insurer — Renewal from previous insurer:
Renewal from previous insurer:
Treatment continued if prior insurer approved and prescriber documents benefit.
Patients with a functional ADAMTS13 inhibitor are excluded from coverage until the provider submits documentation demonstrating the absence of an ADAMTS13 inhibitor prior to initiation of therapy.
Initial Therapy Dosing and Initiation
Initial Therapy Dosing — Initial dosing and clinical initiation rules:
Initial dosing and clinical initiation rules:
Prophylactic dosing; requires meeting initial clinical criteria.
On‑demand regimen; requires meeting acute event documentation.
Continuation and Reauthorization
Continuation Therapy — Reauthorization/continuation requirements:
Reauthorization/continuation requirements:
See Coverage > Reauthorization Criteria.
Provider Action & Documentation Requirements
Prior authorization required
Prior authorization is required. Initial authorization is granted only when the request documents all required clinical criteria including patient age ≥ 2 years, molecular genetic confirmation of biallelic pathogenic ADAMTS13 variants, ADAMTS13 activity < 10% by FRETS‑VWF73 assay, absence of a functional ADAMTS13 inhibitor, trial/failure or contraindication to FFP, and prescribing by or in consultation with a hematologist.
- Must meet all listed clinical criteria for initial authorization (see criteria list).
- Prescriber must be a hematologist or consult with one.
FFP trial required prior to therapy
Before Adzynma is started, the patient must have a documented trial and failure of fresh frozen plasma (FFP) infusion for at least 3 months, or documentation of a contraindication to FFP.
- Document duration of FFP therapy (minimum 3 months) and reason for failure, or provide documentation of contraindication to FFP.
Required clinical documentation
Submit clinical documentation including molecular genetic testing demonstrating biallelic pathogenic ADAMTS13 variants, ADAMTS13 activity result (< 10% by FRETS‑VWF73 assay), documentation of absence of a functional ADAMTS13 inhibitor, provider attestation excluding other TTP‑like diagnoses, and evidence of FFP trial/failure or contraindication; include relevant recent labs and event documentation for prophylaxis or on‑demand requests (platelets, LDH, description of TTP events).
- Molecular genetic testing report showing biallelic pathogenic ADAMTS13 variants.
- ADAMTS13 activity result with assay method (FRETS‑VWF73) and value (< 10%).
- Statement or lab evidence confirming no functional ADAMTS13 inhibitor.
- Provider attestation that other TTP‑like diagnoses are excluded.
- FFP trial/failure documentation or contraindication.
- Recent platelet counts, LDH, and event descriptions as applicable.
Missing diagnostic documentation may lead to denial
Requests that do not document age ≥ 2 years, molecular genetic testing demonstrating biallelic pathogenic ADAMTS13 variants, ADAMTS13 activity < 10% (FRETS‑VWF73), and absence of a functional ADAMTS13 inhibitor may be denied or pended.
- Failure to provide the specified diagnostic evidence risks denial.
FFP trial documentation required to avoid denial
Requests lacking documentation of a trial and failure of FFP infusion for at least 3 months, or lacking documentation of a contraindication to FFP, may be denied or require additional justification.
- Include dates, duration, and outcome of FFP therapy or explicit contraindication details to avoid denial.
Coding & Clinical Thresholds
| J7171 | Injection, ADAMTS13, recombinant-krhn, 10 IU |
Step Therapy / Trial Requirements
| Step | Requirement | Duration / Notes |
|---|---|---|
| 1 | Patient must have trial and failure of fresh frozen plasma (FFP) infusion unless there is a documented contraindication to FFP | At least 3 months (trial/failure) — unless contraindicated |
Site of Care & Administration
Intravenous administration and infusion rate
Adzynma is administered intravenously; infusion for prophylactic dosing is given at a rate of 2–4 mL per minute.
- Infusion route: IV.
- Specified infusion rate: 2–4 mL/min for prophylactic administration.
Background
Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by severe hereditary ADAMTS13 deficiency. Enzyme replacement therapy with Adzynma (recombinant ADAMTS13) is indicated for prophylactic or on‑demand intravenous replacement of ADAMTS13 to prevent or treat acute TTP events.
Definitions
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