CurrentUnitedHealthcarePolicy IEXD00131.03

Adzynma (ADAMTS13, recombinant-krhn) for congenital TTP — Coverage Criteria

Policy governs medical benefit coverage criteria for prophylactic and on-demand treatment with Adzynma in patients with congenital thrombotic thrombocytopenic purpura (cTTP). It applies to UnitedHealthcare plans as specified in member benefit documents.

Policy Summary

PayerUnitedHealthcare

PolicyAdzynma (ADAMTS13, recombinant-krhn) for congenital TTP — Coverage Criteria

Policy CodePolicy IEXD00131.03

Change TypeReference updatearchived prior version

Effective DateJul 1, 2025

Next Review Date

Key ActionObtain prior authorization and document diagnosis, molecular genetic confirmation of an ADAMTS13 mutation, indication, FDA-consistent dosing, and hematology involvement.

SourceLink

POLICY UPDATE CHANGES

Updated References section to reflect the most current information and archived previous policy version IEXD00131.02.

J7171HCPCS code listed

D69.42Diagnosis code listed

12 moProphylactic auth period

3 moOn-demand auth period

Coverage Criteria for Adzynma (ADAMTS13, recombinant-krhn)

Initial prophylactic therapy

Covered when ALL of the following are met for prophylactic therapy (initial):

Initial prophylactic therapy criteria: Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP); ADAMTS13 mutation confirmed by molecular genetic testing; Adzynma is prescribed for routine prophylactic treatment to prevent thrombotic thrombocytopenic purpura; Adzynma dosing is in accordance with the United States Food and Drug Administration approved labeling; prescribed by or in consultation with a hematologist; authorization will be for no more than 12 months.

Continuation prophylactic therapy

Covered when ALL of the following are met for continuation of prophylactic therapy:

Continuation prophylactic therapy criteria: Patient has previously received routine prophylactic treatment with Adzynma; documentation of positive clinical response to Adzynma therapy; Adzynma dosing is in accordance with the United States Food and Drug Administration approved labeling; prescribed by or in consultation with a hematologist; authorization will be for no more than 12 months.

Policy Summary

PayerUnitedHealthcare

PolicyAdzynma (ADAMTS13, recombinant-krhn) for congenital TTP — Coverage Criteria

Policy CodePolicy IEXD00131.03

Change TypeReference updatearchived prior version

Effective DateJul 1, 2025

Next Review Date

Key ActionObtain prior authorization and document diagnosis, molecular genetic confirmation of an ADAMTS13 mutation, indication, FDA-consistent dosing, and hematology involvement.

SourceLink

On This Page

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On-demand (acute) therapy

Covered when ALL of the following are met for on-demand treatment of an acute TTP event:

On-demand acute TTP therapy criteria: Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP); ADAMTS13 mutation confirmed by molecular genetic testing; Adzynma is prescribed for on-demand treatment of an acute thrombotic thrombocytopenic purpura (TTP) event; Adzynma dosing is in accordance with the United States Food and Drug Administration approved labeling; prescribed by or in consultation with a hematologist; authorization will be for no more than 3 months.

There are no additional exclusions listed in this policy beyond the stated coverage criteria. The policy’s coverage stance is covered with criteria, meaning Adzynma will be authorized only when the specific diagnostic, genetic confirmation, dosing, and prescriber requirements are met as set forth in the coverage sections. See the criteria for required documentation and prior authorization limits.

Coverage of Adzynma is linked directly to the underlying diagnosis and to adherence with the FDA-approved dosing and prescribing context. Authorization requires documentation of a diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP), confirmation of an ADAMTS13 mutation by molecular genetic testing, and that dosing is consistent with the United States Food and Drug Administration labeling. Therapy must be prescribed by or in consultation with a hematologist and is subject to the policy’s time limits (see authorization limits).

Applicable Codes

Applicable HCPCS CodesHCPCS

J7171

Injection, ADAMTS13, recombinant-krhn, 10 IU

Applicable Diagnosis CodesICD-10

D69.42

Congenital and hereditary thrombocytopenia purpura

Provider Actions, Prior Authorization, and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for Adzynma. Authorization durations differ by indication: prophylactic therapy approvals are limited to 12 months, while on-demand (acute TTP event) approvals are limited to 3 months. Ensure requests include specialty review by or in consultation with a hematologist.

Authorization duration: prophylaxis — up to 12 months
Authorization duration: on‑demand acute TTP treatment — up to 3 months
Prescriber requirement: hematologist or hematology consultation

Step Therapy

Step / Sequencing Considerations

Step or sequencing considerations may affect therapy choice. When clinically appropriate, plasma‑based therapies (e.g., FFP) have been used historically for prophylaxis and on‑demand treatment; ADAMTS13, recombinant‑krhn (Adzynma) demonstrated efficacy in trials and may be used instead of or following plasma‑based therapies depending on clinical context and member plan rules.

Plasma-based therapies (FFP, FVIII‑VWF concentrate) are alternative options and were used in study comparator arms
Consider prior use of plasma-based therapy and clinical response when sequencing to Adzynma
No mandated step requiring plasma before Adzynma is specified here — follow member benefit and medical necessity criteria

Documentation Required

Required Documentation for Authorization

Document and submit key clinical information to support medical necessity determinations: diagnosis, confirmatory genetic testing, indication (prophylactic vs on‑demand), dosing per FDA labeling, prior treatment history, and treating specialist involvement.

Diagnosis: congenital thrombotic thrombocytopenic purpura (cTTP) (ICD-10 D69.42)
Molecular genetic confirmation of ADAMTS13 mutation
Indication: routine prophylactic treatment or on‑demand acute TTP event
Dosing consistent with FDA‑approved labeling
Documentation of prescribing clinician (hematologist) or consultation
For continuation requests: documentation of prior Adzynma use and positive clinical response

Denial Risk

Denial Triggers and Documentation Risks

Common reasons authorizations may be denied include missing or incomplete diagnostic or genetic confirmation, requests inconsistent with FDA‑approved dosing or indication, lack of hematology involvement, or insufficient documentation of prior therapy/response when requesting continuation.

Missing diagnosis of cTTP
No molecular genetic confirmation of ADAMTS13 mutation
Requested use not consistent with prophylactic vs on‑demand indications
Dosing that is not in accordance with FDA labeling
Prescriber not a hematologist and no documented consultation
For continuation: no documentation of prior Adzynma use or lack of documented clinical benefit

Background

ADAMTS13 is a plasma zinc metalloprotease that regulates von Willebrand factor by cleaving large multimers, thereby reducing platelet binding and microthrombus formation. Recombinant ADAMTS13 (Adzynma) provides enzyme replacement in patients with congenital deficiency and is indicated for both routine prophylactic and on‑demand treatment of acute TTP events in adults and pediatric patients with cTTP.

Key Definitions

ADAMTS13 — definition

DefinitionADAMTS13 is a plasma zinc metalloprotease that cleaves large and ultra‑large von Willebrand factor (VWF) multimers into smaller units, reducing VWF platelet‑binding properties and the propensity to form microthrombi.

Molecular roleRegulates VWF activity by proteolytic cleavage of multimers to limit platelet aggregation and microthrombus formation.

Endogenous vs recombinantAdzynma is a recombinant form of the endogenous ADAMTS13 enzyme.

Adzynma — FDA indication

Approved indicationAdzynma (ADAMTS13, recombinant‑krhn) is indicated for prophylactic or on‑demand enzyme replacement therapy in adult and pediatric patients with congenital thrombotic thrombocytopenic purpura (cTTP).

Product typeHuman recombinant rADAMTS13 (A disintegrin and metalloproteinase with thrombospondin motifs 13).

PopulationIndicated for both adult and pediatric patients with congenital TTP (cTTP).

Policy Revision History

2025-07-01policy effective / revisionLatest

Updated References section to reflect the most current information and archived previous policy version IEXD00131.02.