CurrentAnthemPolicy CC-0252

Adzynma (ADAMTS13, recombinant-krhn) — Medical Drug Clinical Criteria

Medical necessity criteria, coding, and review guidance for coverage of Adzynma (recombinant ADAMTS13) for treatment and prevention of congenital thrombotic thrombocytopenic purpura (cTTP) under the medical benefit.

Policy Summary

PayerAnthem

PolicyAdzynma (ADAMTS13, recombinant-krhn) — Medical Drug Clinical Criteria

Policy CodePolicy CC-0252

Change TypeReviewed — no material change

Effective Date07/01/2025

Next Review Date05/16/2026

Key ActionObtain prior authorization with documentation of diagnosis, molecular genetic testing for ADAMTS13 mutation, ADAMTS13 activity <10%, and relevant platelet/LDH labs.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

FDA 11/9/2023approval date noted

J7171HCPCS

<10%ADAMTS13 threshold

<100k/50% dropon-demand platelet

>2x ULNLDH criterion

Coverage Criteria for Adzynma (recombinant ADAMTS13)

Initial Therapy (On-demand or Prophylactic)

Initial requests for Adzynma may be approved if the following criteria are met:

Initial approval for on-demand treatment: I. Diagnosis of severe congenital thrombotic thrombocytopenic purpura (cTTP); AND II. Individual is using for on-demand treatment; AND III.A molecular genetic testing showing mutation in the ADAMTS13 gene; AND III.B ADAMTS13 activity testing showing less than 10% of normal ADAMTS13 activity; AND IV. Documentation of a >=50% drop in platelet count from baseline OR platelet count < 100,000/microliter; AND V. Lactate dehydrogenase (LDH) > 2x baseline OR > 2x upper limit of normal (ULN)See individual lab criteria

Supports on-demand approval per pivotal trials (NCT03393975, NCT04683003)

Initial approval for prophylactic treatment: I. Diagnosis of severe congenital thrombotic thrombocytopenic purpura (cTTP); AND II. Individual is using for prophylactic treatment; AND III.A molecular genetic testing showing mutation in the ADAMTS13 gene; AND III.B ADAMTS13 activity testing showing less than 10% of normal ADAMTS13 activity; AND IX. Documentation of platelet count > 100,000/microliter; AND X. Lactate dehydrogenase (LDH) <= 2x upper limit of normal (ULN)

Policy Summary

PayerAnthem

PolicyAdzynma (ADAMTS13, recombinant-krhn) — Medical Drug Clinical Criteria

Policy CodePolicy CC-0252

Change TypeReviewed — no material change

Effective Date07/01/2025

Next Review Date05/16/2026

Key ActionObtain prior authorization with documentation of diagnosis, molecular genetic testing for ADAMTS13 mutation, ADAMTS13 activity <10%, and relevant platelet/LDH labs.

SourceLink

On This Page

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See individual lab criteria

Supports prophylactic approval per pivotal trials (NCT03393975, NCT04683003)

Continuation Therapy

Continuation requests for Adzynma may be approved if the following criteria are met:

Continuation criteria: I. For on-demand use: documentation that platelets increase to at least 150,000/microliter OR increase by 25% from baseline; OR II. For prophylactic use: documentation of decreased number of TTP eventsClinical response (platelet rise) or reduction in TTP events

Evidence of clinical benefit required for ongoing therapy

Not Medically Necessary / Exclusions

Not approved indications: Requests for Adzynma are not approved for individuals diagnosed with acquired idiopathic or secondary forms of thrombotic thrombocytopenic purpura; OR when the above criteria are not met (all other indications)N/A

Policy restricts coverage to congenital (hereditary) TTP only

Requests for Adzynma (ADAMTS13, recombinant-krhn) will not be approved for individuals diagnosed with acquired idiopathic or secondary thrombotic thrombocytopenic purpura (TTP). This policy is limited to treatment of severe congenital TTP (cTTP) confirmed by molecular genetic testing for an ADAMTS13 mutation and ADAMTS13 activity < 10% of normal as specified in the coverage criteria.

If the specific coverage criteria for Adzynma are not met, requests for treatment with Adzynma are not medically necessary. This includes any use outside the defined indications and required biomarker/laboratory thresholds (for example, absence of documented ADAMTS13 mutation or ADAMTS13 activity ≥ 10%, or failure to meet the platelet and LDH criteria for on‑demand or prophylactic use).

Coding and Laboratory Thresholds

HCPCSHCPCSCovered

J7171

Injection, ADAMTS13, recombinant-krhn, 10 IU [Adzynma]

ICD-10 DiagnosisICD-10

D69.42

Congenital and hereditary thrombocytopenia purpura

inv-08: ADAMTS13 activity — biomarker threshold display (< 10%)

ThresholdADAMTS13 activity < 10% of normal (measured by FRETS-VWF73 assay)

Required forBoth initial (on-demand and prophylactic) and continuation approvals

Evidence sourceMolecular genetic testing plus ADAMTS13 activity <10% required per pivotal trials (NCT03393975, NCT04683003)

inv-09: Platelet count (on-demand) — show 50% drop OR < 100,000/microliter threshold.

On-demand platelet criterion50% or greater drop from baseline OR platelet count < 100,000/microliter

When requiredRequired for initial on‑demand treatment requests

DocumentationProvide platelet counts demonstrating drop or absolute value at time of request

inv-10: LDH (on-demand) — > 2x baseline OR > 2x ULN threshold.

LDH threshold (on‑demand)> 2 times baseline OR > 2 times upper limit of normal (ULN)

When requiredRequired for initial on‑demand treatment requests

DocumentationProvide LDH laboratory values showing elevation relative to baseline or ULN

inv-11: Platelet count (prophylaxis) — threshold > 100,000/microliter.

Prophylaxis platelet criterion> 100,000/microliter

When requiredRequired for initial prophylactic treatment requests

DocumentationProvide platelet count demonstrating value >100,000/microliter at time of request

inv-12: LDH (prophylaxis) — threshold <= 2x ULN.

LDH threshold (prophylaxis)<= 2 times upper limit of normal (ULN)

When requiredRequired for initial prophylactic treatment requests

DocumentationProvide LDH laboratory values showing <=2x ULN

Provider Actions, Authorization, and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization is required for Adzynma (ADAMTS13, recombinant-krhn). Providers must obtain prior authorization before initiating therapy for either on-demand or prophylactic use in individuals with severe congenital thrombotic thrombocytopenic purpura (cTTP).

Submit prior authorization for Adzynma (J7171) before treatment initiation
Indicate whether request is for on-demand (acute) or prophylactic therapy
Include diagnosis code D69.42 and relevant clinical notes

Note

Provider Actions

Providers should document clinical status and intended use at time of request and follow-up documentation for continuation requests. Prior authorization requests should clearly state the indication (on-demand vs prophylactic) and reference objective laboratory and genetic results supporting severe congenital TTP.

State if request is Initial or Continuation
For continuation, provide documentation of clinical response (see documentation callout)

Documentation Required

Required Documentation

Required documentation must be provided with the prior authorization request. Include the following objective items in the record:

Molecular genetic testing demonstrating a pathogenic mutation in the ADAMTS13 gene
ADAMTS13 activity testing showing < 10% of normal activity
Platelet counts (baseline and current) demonstrating either a ≥50% drop or platelet count < 100,000/µL for on-demand treatment; for prophylaxis, documentation of platelet count > 100,000/µL as applicable
Lactate dehydrogenase (LDH) values showing >2× baseline or >2× ULN for on-demand treatment, or LDH <2× ULN for prophylactic candidates
Clinical notes specifying whether therapy is on-demand or prophylactic and history of prior TTP events (for prophylactic use)

Denial Risk

Denial Conditions

Requests will be denied if the criteria for severe congenital TTP are not met, if required documentation is missing, or if the diagnosis is acquired/idiopathic or secondary TTP rather than congenital TTP.

Denial when ADAMTS13 activity is ≥ 10% or no genetic mutation in ADAMTS13 is documented
Denial for acquired or secondary forms of TTP (non-congenital)
Denial for incomplete prior authorization submissions or missing lab/genetic documentation
Denial when clinical/platelet/LDH criteria for the requested treatment intent (on-demand vs prophylactic) are not satisfied

Required Biomarkers and Genetic Testing

inv-19: ADAMTS13 activity — high-importance biomarker requirement (< 10% of normal).

Biomarker requirementADAMTS13 activity < 10% of normal (measured by FRETS‑VWF73 assay)

Required forBoth initial (on‑demand or prophylactic) and continuation approvals

Supporting evidenceReferenced pivotal studies NCT03393975 and NCT04683003 used these criteria

inv-20: Molecular genetic testing for ADAMTS13 mutation — presence of ADAMTS13 gene mutation required.

RequirementMolecular genetic testing demonstrating a pathogenic ADAMTS13 gene mutation is required

Applies toBoth initial on‑demand and prophylactic treatment requests

DocumentationProvide molecular genetic testing results as part of prior authorization submission

Definitions

inv-18: Definition of severe hereditary ADAMTS13 deficiency by molecular genetic testing and ADAMTS13 activity (<10%, FRETS-VWF73 assay).

DefinitionSevere hereditary ADAMTS13 deficiency defined as presence of ADAMTS13 gene mutation on molecular genetic testing and ADAMTS13 activity <10% (measured by FRETS‑VWF73 assay)

Assay specifiedFRETS‑VWF73 assay used to measure ADAMTS13 activity

Clinical contextDefinition was used to enroll subjects in pivotal trials and to confirm diagnosis in medical history or screening

Background

Adzynma (ADAMTS13, recombinant-krhn) is a recombinant form of the ADAMTS13 enzyme intended to replace deficient ADAMTS13 activity in patients with congenital thrombotic thrombocytopenic purpura (cTTP). Pivotal clinical trials (NCT03393975, NCT04683003) enrolled individuals with confirmed hereditary ADAMTS13 deficiency defined by molecular genetic testing showing an ADAMTS13 gene mutation and ADAMTS13 activity < 10% of normal, and excluded acquired or secondary forms of TTP. The policy’s initial and continuation criteria mirror the trial entry criteria and reported clinical response measures used in those studies.

Revision History

2025-05-16Annual review / coding updateLatest

Annual review with coding review; updated description for HCPCS J7171.

2024-05-17Annual review / coding changes

Annual review noting no clinical changes; effective 2024-07-01 removed HCPCS J3590, J9399, C9167 and added HCPCS J7171 and ICD-10-CM D69.42.

2023-11-17Policy creation / initial coding

Policy created with clinical criteria for Adzynma and initial coding additions (HCPCS J3590, C9399); later noted additions/effective dates for C9167 on 2024-04-01.

2023-11-09Regulatory approval

FDA approved Adzynma (ADAMTS13, recombinant-krhn).