CurrentAvMedPolicy N/A

Adzynma (ADAMTS13 recombinant-krhn) — Prior Authorization for congenital TTP (cTTP)

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.

Policy Summary

PayerAvMed

PolicyAdzynma (ADAMTS13 recombinant-krhn) — Prior Authorization for cTTP

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionProvider must complete and sign the prior authorization request and submit documentation confirming biallelic ADAMTS13 pathogenic variants and ADAMTS13 activity <10%.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2 yearsminimum member age for therapy

6 monthsinitial auth length (prophylaxis)

3 monthsinitial auth length (on-demand)

12 monthsreauthorization interval

up to 4 attacks/4 weeksquantity covers

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Coverage Criteria for Adzynma (cTTP)

Initial Authorization (cTTP)

Covered when ALL of the following are met

Initial Authorization - general: Member is ≥2 years of age; prescribed by or in consultation with a hematologist/oncologist or a provider knowledgeable in genetic hematologic diseases; confirmed diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP) due to inherited ADAMTS13 deficiency; submission of molecular genetic testing documenting biallelic pathogenic variants in the ADAMTS13 gene; ADAMTS13 activity <10% by FRETS-VWF73 assay (patients currently receiving prophylactic plasma infusion therapy may exceed 10% at start of therapy if clearly documented); laboratory documentation confirming absence of anti-ADAMTS13 IgG inhibitory autoantibodies; no diagnosis of other cTTP-like disorders (e.g., acquired/immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia-ITP, Evans Syndrome); no medical history or presence of a functional ADAMTS13 inhibitor; and no known sensitivity to hamster protein.

All listed checklist items required.

Treatment Intent (Prophylaxis or On-Demand)

Provider selects ONE treatment intent and meets associated documentation

Treatment intent: Prophylactic therapy: provider has submitted documentation of ≥1 prior TTP event or is currently receiving prophylactic plasma infusion therapy (patients on plasma prophylaxis should discontinue routine plasma after achieving therapeutic response). OR On-demand therapy: patient is at risk of disease exacerbation.

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)

Reauthorization for prophylaxis: Provider attests member has NOT reported unacceptable toxicity (e.g., severe hypersensitivity/anaphylaxis, severe abdominal pain, diarrhea, headache); member has NOT developed neutralizing antibodies to ADAMTS13; member has responded to therapy compared to pre-treatment baseline as evidenced by reduction/absence of acute or sub-acute TTP events or relevant laboratory markers.

Prophylaxis reauthorization interval: 12 months.

Reauthorization for on-demand: Provider attests member has NOT reported unacceptable toxicity; member has NOT developed neutralizing antibodies to ADAMTS13; member responded to an acute TTP event with therapy as evidenced by improvement in thrombocytopenia (platelet count ≥150,000/μL or within 25% of baseline) or improvement in MAHA (LDH ≤1.5× baseline or ≤1.5× ULN).

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

Covered HCPCS Code (Medical)HCPCSCovered

C9167

Adzynma (ADAMTS13 recombinant-krhn) (Medical)

NDCsNDC

64764-0135-xx

NDC for Adzynma 1500 IU single-dose vial

Dosing and quantity limits — prophylaxis and on‑demand

Prophylaxis dosing4500 IU every other week (40 units/kg/dose once every other week); may increase frequency to once weekly

Prophylaxis vial quantity (NDC units)500 IU vial: 2 vials every other week; 1500 IU vial: 3 vials every other week

On‑demand dosing — Day 14500 IU (40 units/kg once)

On‑demand dosing — Day 23000 IU (20 units/kg once)

On‑demand dosing — Days 3–71500 IU once daily for 5 more days (15 units/kg/dose once daily); continue for 2 days after acute event has resolved

On‑demand vial quantity (NDC units)500 IU vial: 2 vials daily for 7 days; 1500 IU vial: 3 vials on day 1, 2 vials on day 2, then 1 vial daily for 5 more days

Provider Requirements and Authorization Process

Prior Authorization

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)

Note

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)

Documentation Required

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)

Denial Risk

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

Key Definitions

cTTP due to inherited ADAMTS13 deficiency — definition and diagnostic thresholds

Definition of cTTP due to inherited ADAMTS13 deficiencyConfirmed by biallelic pathogenic variants in ADAMTS13 and ADAMTS13 activity <10% by FRETS‑VWF73 assay, with absence of anti‑ADAMTS13 IgG inhibitory autoantibodies

Age criterionMember is at least 2 years of age or older

Provider qualificationPrescribed by or in consultation with a hematologist/oncologist or a provider knowledgeable in genetic hematologic diseases

ExclusionsNo diagnosis of acquired/immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia (ITP), or Evans Syndrome

Acute TTP event — laboratory definition

Platelet criterionDrop in platelet count ≥50% of baseline or absolute platelet count <100,000/μL

LDH criterionElevation of lactate dehydrogenase (LDH) >2× baseline or >2× upper limit of normal (ULN)

Combined lab‑based event definitionAn acute TTP event is defined by both the platelet drop criterion and the LDH elevation criterion

OpenPayer

Adzynma (ADAMTS13 recombinant-krhn) — Prior Authorization for congenital TTP (cTTP)

Coverage Criteria for Adzynma (cTTP)

Billing Codes, NDCs, and Dosing Limits

Provider Requirements and Authorization Process

Genetic and Laboratory Requirements

Key Definitions

Background on Adzynma and Indication