CurrentViva HealthPolicy IC-0736

Adzynma (recombinant ADAMTS13) coverage for cTTP

Policy governs medical coverage, authorization, dosing, and renewal criteria for Adzynma (recombinant ADAMTS13) when used to treat congenital thrombotic thrombocytopenic purpura (cTTP) for Viva Health members.

Policy Summary

PayerViva Health

PolicyAdzynma (recombinant ADAMTS13) coverage for cTTP

Policy CodePolicy IC-0736

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization specifying prophylactic (initial 6 months, renewable annually) or on‑demand therapy (initial 3 months, renewable) and document genetic testing confirming biallelic ADAMTS13 pathogenic variants plus ADAMTS13 activity <10%.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2 yearsmin age

1800max units/28d

40 IU/kgprophylactic dose

J7171HCPCS

D69.42dx code

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Initial Approval

Covered when ALL of the following are met for initial approval

Initial Approval Criteria: Patient is at least 2 years of age.

Minimum age requirement.

Initial Approval Criteria: Patient has not been diagnosed with other cTTP-like disorders (e.g., acquired TTP, immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, Evans Syndrome).

Exclusion for alternate diagnoses.

Initial Approval Criteria: Patient does not have a medical history or the presence of functional ADAMTS13 inhibitors prior to the start of therapy.

Presence of functional inhibitors excludes eligibility.

Initial Approval Criteria: Patient does not have a known sensitivity to hamster protein.

Safety exclusion.

Initial Approval Criteria: Documented diagnosis of severe hereditary ADAMTS13 deficiency confirmed by molecular genetic testing demonstrating biallelic pathogenic ADAMTS13 variants.

Diagnostic genetic testing required.

Initial Approval Criteria: ADAMTS13 activity < 10% measured by FRETS-VWF73 assay.< 10%

Laboratory threshold for biochemical confirmation (patients on prophylactic plasma may exceed 10% at therapy start).

Therapy intent (one of)

Prophylactic therapy: Treatment will be used as prophylactic therapy AND patient has a history of at least one TTP event or is currently receiving prophylactic plasma infusion therapy.
Patients receiving routine prophylactic plasma-based therapies should discontinue routine use after achieving therapeutic response.
On-demand therapy: Treatment will be used as on-demand therapy and patient is at risk of disease exacerbation.
On-demand use for acute risk situations.

Renewal Criteria

Renewal criteria — coverage may be renewed when ALL of the following are met

Renewal Criteria: Patient continues to meet the universal and indication-specific relevant criteria identified for initial approval.

Ongoing eligibility requirement.

Renewal Criteria: Absence of unacceptable toxicity from the drug (examples include severe hypersensitivity or anaphylactic reactions).

Serious adverse events may preclude renewal.

If symptomatic: Improvement in signs and symptoms of disease (e.g., neurological symptoms, renal dysfunction, TTP-related pain).
Clinical improvement required for symptomatic patients.
If prophylaxis:

Authorization Durations

Authorization durations

Authorization Durations: Prophylaxis: initial coverage for 6 months, may be renewed annually. On-demand: initial coverage for 3 months, eligible for renewal.

Authorizations will consider cumulative on‑hand supply to permit treatment of up to 4 acute attacks per 4 weeks for the duration of the authorization.

Patients with disorders that mimic congenital thrombotic thrombocytopenic purpura (cTTP) are excluded from coverage under this policy. Examples include acquired/immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, and Evans Syndrome. Coverage is limited to the FDA‑approved indication for cTTP with documentation specific to hereditary ADAMTS13 deficiency.

Therapy is not supported for patients with evidence of a functional ADAMTS13 inhibitor present prior to initiation. Presence of functional inhibitors excludes eligibility under these criteria and should be documented in the medical record prior to authorization.

Billing, Diagnosis, and Test Codes

Covered HCPCS CodesHCPCSCovered

J7171

Injection, adamts13, recombinant-krhn, 10 iu; 1 billable unit = 10 units

NDCNDC

64764-0130-xx	Adzynma 500 IU single-dose vial for injection
64764-0135-xx	Adzynma 1500 IU single-dose vial for injection

Covered Diagnosis CodesICD-10Covered

D69.42

Congenital and hereditary thrombocytopenia purpura

inv-09: ADAMTS13 activity (FRETS-VWF73) — documented threshold value for eligibility

ThresholdADAMTS13 activity < 10% as measured by the FRETS-VWF73 assay

Assay noteFRETS-VWF73 specified; patients on prophylactic plasma may exceed 10% at therapy start

Context of useRequired for diagnosis of severe hereditary ADAMTS13 deficiency prior to coverage

inv-10: HCPCS quantity limit — maximum billable units every 28 days

Maximum billable units1800 billable units every 28 days

Unit definitionHCPCS units as billed under J7171 (1 billable unit = 10 IU) per coding table

Authorization impactQuantity limit constrains prior authorizations and renewals (supply planning up to 4 acute attacks per 4 weeks)

Provider Requirements, Prior Authorization, and Documentation

Prior Authorization

Prior Authorization and Quantity Limits

Prior Authorization Required. Prior authorization is required before initiating therapy. Approvals and duration depend on intended use: prophylactic approvals are granted for 6 months (with annual renewal thereafter) and on‑demand approvals are granted for 3 months (eligible for renewal). Quantity limits: maximum 1800 billable units (HCPCS unit) every 28 days. When authorizing on‑demand therapy, the cumulative amount of medication the patient will have on hand to treat acute TTP events will be considered — authorizations should provide sufficient quantity to treat up to 4 acute attacks per 4 weeks for the duration of the authorization.

Prophylaxis: initial approval 6 months; renew annually thereafter.
On‑demand: initial approval 3 months; eligible for renewal.
Quantity limit: 1800 billable units per 28 days (HCPCS unit).
On‑demand authorizations should account for supply to treat up to 4 acute attacks per 4 weeks.

Denial Risk

Eligibility‑Related Denial Triggers

Denial risk — do not approve if any of the following apply: patient is younger than 2 years of age; diagnosis is consistent with other TTP‑like disorders (for example, acquired/immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, Evans syndrome); or there is a medical history or presence of functional ADAMTS13 inhibitors prior to the start of therapy.

Age < 2 years is a trigger for denial.
Diagnoses consistent with acquired/immune TTP or other TTP‑like disorders are exclusionary.
Presence or history of functional ADAMTS13 inhibitors prior to therapy is exclusionary.

Documentation Required

Diagnostic Documentation

Documentation Required. Prior authorization requests must include diagnostic confirmation of congenital TTP: molecular genetic testing demonstrating biallelic pathogenic ADAMTS13 variants and laboratory confirmation of ADAMTS13 activity <10% measured by the FRETS‑VWF73 assay. Note: patients currently receiving prophylactic plasma infusion therapy may have ADAMTS13 activity >10% at therapy start; documentation should note prior plasma‑based therapy and, if applicable, that routine prophylactic plasma therapies will be discontinued after achieving a therapeutic response.

Molecular genetic testing confirming biallelic pathogenic ADAMTS13 variants.
ADAMTS13 activity < 10% by FRETS‑VWF73 assay (document assay type).
If patient is on prophylactic plasma therapy, include note — routine prophylactic plasma‑based therapies should be discontinued after therapeutic response is achieved.

Definitions and Clinical Thresholds

inv-16: Severe hereditary ADAMTS13 deficiency — definition requiring molecular genetic confirmation of biallelic pathogenic ADAMTS13 variants

DefinitionConfirmed by molecular genetic testing documenting biallelic pathogenic variants in ADAMTS13

Supporting biochemical criterionAssociated requirement: ADAMTS13 activity <10% by FRETS-VWF73 assay

Clinical implicationRequired documentation for coverage of recombinant ADAMTS13 (Adzynma) for cTTP

inv-17: TTP event definitions — acute and sub-acute TTP event clinical thresholds (platelet drop, LDH elevations), and related criteria

Acute TTP event (prophylaxis outcome)Defined as a drop in platelet count ≥50% of baseline or platelet <100,000/μL plus LDH elevation >2× baseline or >2× ULN

Sub-acute TTP eventDefined as thrombocytopenia or microangiopathic hemolytic anemia with organ-specific signs/symptoms (e.g., renal dysfunction, neurologic symptoms, fever, fatigue, abdominal pain)

On-demand response criteriaImprovement in thrombocytopenia (platelet drop ≥25% of baseline or platelet <150,000/μL) or improvement in MAHA (LDH >1.5× baseline or >1.5× ULN)

OpenPayer

Adzynma (recombinant ADAMTS13) coverage for cTTP

Coverage Criteria for Adzynma (recombinant ADAMTS13)

Billing, Diagnosis, and Test Codes

Provider Requirements, Prior Authorization, and Documentation

Background and Clinical Context

Definitions and Clinical Thresholds