Adzynma (ADAMTS13 recombinant-krhn) — Prior Authorization for congenital TTP (cTTP)
Customize your policy alerts
Sign up for all AvMed policy alerts
Know when AvMed releases new policies or updates existing guidance.
Monitor payer policy activity
Prior authorization and clinical criteria for medical (clinic/infusion) use of Adzynma for congenital thrombotic thrombocytopenic purpura (cTTP) for AvMed members; applies to providers requesting coverage of prophylactic or on-demand therapy.
No material clinical or coverage changes in this revision.
Coverage Criteria for Adzynma (cTTP)
Initial Authorization (cTTP)
Covered when ALL of the following are met
All listed checklist items required.
Treatment Intent (Prophylaxis or On-Demand)
Provider selects ONE treatment intent and meets associated documentation
Provider must specify prophylaxis or on-demand on PA form. Cumulative on-hand quantity to cover up to 4 acute attacks per 4 weeks will be considered for authorization.
Reauthorization Criteria
Reauthorization criteria (must all be met for renewal)
Prophylaxis reauthorization interval: 12 months.
On-demand reauthorization interval: 3 months.
Members with acquired/immune TTP or other primary thrombotic microangiopathies are not eligible for this congenital TTP–specific coverage pathway. Also excluded are members with immune thrombocytopenia (ITP) or Evans Syndrome. The policy requires documentation excluding a diagnosis of these alternate TMA/platelet-disorder conditions as part of the authorization checklist.
The use of manufacturer samples to start therapy does not satisfy preauthorization or step-edit requirements. Providers initiating treatment with samples must still submit the required PA request and supporting documentation; prior therapies will be verified through pharmacy paid claims or submitted chart notes.
Billing Codes, NDCs, and Dosing Limits
| C9167 | Adzynma (ADAMTS13 recombinant-krhn) (Medical) |
| 64764-0135-xx | NDC for Adzynma 1500 IU single-dose vial |
Provider Requirements and Authorization Process
Prior Authorization Required
Prior authorization is required for Adzynma (ADAMTS13 recombinant-krhn) (C9167) when billed as a medical benefit. Complete the Medical Prior Authorization/Step-Edit Request fully — the prescribing physician must sign and print name (preprinted stamps not valid). Fax requests to 1-877-535-1391. Missing, incomplete, incorrect, or illegible information (including phone/fax numbers) may delay review; authorization can be delayed or denied if supporting documentation is not provided.
- Drug: Adzynma (ADAMTS13 recombinant-krhn) (C9167) (Medical)
- Fax: 1-877-535-1391
- Prescribing physician signature required (no preprinted stamps)
Samples and Verification
Samples do NOT satisfy prior authorization or step-edit requirements and do not count toward prior-therapy verification. For urgent reviews, contact AvMed Pre-Authorization Department when lack of treatment could seriously jeopardize life, health, or ability to regain maximum function. Previous therapies will be verified via pharmacy paid claims or submitted chart notes.
- Use of samples to initiate therapy does not meet step edit/preauthorization criteria.
- Specialty Pharmacy: Proprium Rx (if applicable)
Required Clinical Documentation
Providers must supply complete clinical documentation with initial and reauthorization requests. For initial authorization include: molecular genetic testing documenting biallelic pathogenic ADAMTS13 variants; ADAMTS13 activity measured by FRETS‑VWF73 showing <10% (note exceptions if on prophylactic plasma infusion therapy with documented regimen); laboratory confirmation that anti‑ADAMTS13 IgG inhibitory autoantibodies are NOT present; documentation excluding other cTTP‑like disorders; confirmation of no functional ADAMTS13 inhibitor; documentation of no known sensitivity to hamster protein; patient age (≥2 years) and weight when applicable; and that treatment is prescribed by or in consultation with a hematologist/oncologist or provider experienced in genetic hematologic diseases. For reauthorization include documentation of treatment response and absence of unacceptable toxicity or development of neutralizing antibodies — examples of response include reduction/absence of acute or sub‑acute TTP events or improvement in platelet count and LDH per criteria in the authorization form. All lab results, diagnostics, and chart notes supporting each checked item must be provided or the request may be denied.
- Initial: molecular genetic testing showing biallelic pathogenic ADAMTS13 variants
- Initial: ADAMTS13 activity <10% by FRETS‑VWF73 assay (exceptions noted)
- Initial: anti‑ADAMTS13 IgG inhibitors NOT present
- Initial: exclusion of other cTTP‑like disorders and no functional ADAMTS13 inhibitor
- Initial: age ≥2 years, provider specialty: hematology/oncology or equivalent
- Reauthorization: documentation of clinical response (reduction/absence of TTP events or lab improvement)
- Reauthorization: no unacceptable toxicity and no neutralizing antibodies
- All supporting lab results, diagnostics, and chart notes must be submitted
Incomplete Documentation Delays or Denies Request
Incomplete, missing, illegible, or inconsistent documentation may delay review or result in denial of the prior authorization request. Ensure all required fields on the request form are completed (drug name/form/strength, dosing schedule, length of therapy, diagnosis and ICD code if applicable, weight and date obtained when applicable), and attach all supporting laboratory results, genetic testing reports, progress notes, and prior therapy verification.
- Authorization may be delayed or denied if information on the form is incomplete, incorrect, or illegible
- Include drug name/form/strength, dosing schedule, therapy length, diagnosis/ICD, weight/date, and provider contact info
- Attach lab results, genetic test reports, progress notes, and prior therapy verification
Genetic and Laboratory Requirements
Key Definitions
Background on Adzynma and Indication
Adzynma (recombinant ADAMTS13) is indicated for replacement therapy in congenital thrombotic thrombocytopenic purpura (cTTP) caused by inherited ADAMTS13 deficiency. Clinical use includes both prophylactic replacement to prevent TTP events and on‑demand treatment for acute exacerbations. Initial authorization requires documentation of biallelic ADAMTS13 pathogenic variants, an ADAMTS13 activity of <10% by FRETS‑VWF73 assay, absence of anti‑ADAMTS13 inhibitory IgG, and provider attestation that the member meets age and specialist/consultation requirements. Prophylaxis approvals are issued for 6 months (with annual renewal possible); on‑demand approvals are issued for 3 months per approval.
OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.