CurrentColorado Rocky Mountain Health PlansPolicy 2025D0131D

Adzynma (ADAMTS13, Recombinant-Krhn) — Coverage Criteria for Congenital TTP

Defines medical necessity criteria, prior authorization limits, and coding for prophylactic and on‑demand use of Adzynma in patients with congenital thrombotic thrombocytopenic purpura (cTTP); applies to UnitedHealthcare commercial/Community Plan benefit administration.

Policy Summary

PayerColorado Rocky Mountain Health Plans

PolicyAdzynma (ADAMTS13, Recombinant-Krhn) — Coverage Criteria for Congenital TTP

Policy CodePolicy 2025D0131D

Change TypeNo material changes

Effective DateJul 1, 2025

Next Review Date

Key ActionObtain prior authorization and document ADAMTS13 mutation by molecular genetic testing with hematologist involvement; authorizations limited to up to 12 months for prophylaxis or up to 3 months for on‑demand treatment.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsProphylaxis auth

3 monthsOn‑demand auth

J7171HCPCS

D69.42Diagnosis

Coverage Criteria for Adzynma (ADAMTS13, recombinant-krhn)

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Prophylactic (initial therapy) — Medical necessity

Covered when ALL of the following are met

Prophylactic initial therapy criteria: Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP); ADAMTS13 mutation is confirmed by molecular genetic testing; Adzynma is being prescribed for routine prophylactic treatment to prevent thrombotic thrombocytopenic purpura (TTP) events; 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.

Prophylactic (continuation therapy) — Medical necessity

Covered when ALL of the following are met

Prophylactic continuation 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.

On‑demand (acute TTP event) — Medical necessity

Covered when ALL of the following are met

On‑demand acute therapy criteria: Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP); ADAMTS13 mutation is confirmed by molecular genetic testing; Adzynma is being 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.

Coverage determinations for Adzynma are subject to the member’s specific benefit plan document and any applicable federal or state mandates. Some Certificates of Coverage permit experimental/investigational therapies for life‑threatening conditions under defined circumstances, and certain state mandates may require coverage for off‑label medication use. When such mandates apply, they take precedence over the benefit document or this policy. Providers and reviewers should consult the member’s plan and any relevant mandates when applying the clinical criteria in this policy.

Provider Actions, Authorization, and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for both prophylactic and on-demand use of Adzynma (ADAMTS13, recombinant-krhn). Authorizations for prophylactic therapy will be granted for no more than 12 months. Authorizations for on‑demand (acute) treatment will be granted for no more than 3 months.

Authorization duration for prophylaxis: up to 12 months
Authorization duration for on‑demand (acute) treatment: up to 3 months
Prescribing or consultation requirement: hematologist

Step Therapy

Step Therapy / Comparators

Policy compares ADAMTS13, recombinant-krhn to plasma‑based therapies (e.g., fresh frozen plasma and factor concentrates) as the clinical comparator in trials. There is no step‑therapy requirement mandating trial of plasma‑based therapies before coverage of Adzynma when the clinical criteria are met.

Comparators referenced: plasma‑based therapies (FFP, FVIII‑VWF concentrates)
No step therapy required when criteria are satisfied

Documentation Required

Required Clinical Documentation

To support authorization, submit documentation including diagnosis, molecular genetic confirmation of an ADAMTS13 mutation, indication (prophylactic vs on‑demand), dosing consistent with FDA labeling, and documentation that the prescribing clinician is a hematologist or that care is in consultation with a hematologist.

Required: diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP)
Required: molecular genetic testing confirming ADAMTS13 mutation
Required: indication specified (routine prophylaxis or on‑demand acute treatment)
Required: dosing consistent with FDA‑approved labeling
Required: prescriber is a hematologist or documentation of hematology consultation

Denial Risk

Denial Triggers for Prophylactic Therapy

Denial may be issued for prophylactic therapy requests if key criteria are missing or not met, including absence of molecular genetic confirmation of an ADAMTS13 mutation, lack of documentation that therapy is for routine prophylaxis, dosing inconsistent with FDA labeling, or absence of hematologist involvement.

Missing molecular genetic confirmation of ADAMTS13 mutation
Request does not document routine prophylactic intent to prevent TTP events
Dosing not consistent with FDA‑approved labeling
Prescriber is not a hematologist and no consultation with a hematologist is documented

Denial Risk

Denial Triggers for On‑Demand Therapy

Denial may be issued for on‑demand (acute) treatment requests if required elements are absent or criteria are not met. Common denial triggers include missing molecular genetic confirmation of ADAMTS13 mutation, missing documentation that the request is for treatment of an acute TTP event, dosing not aligned with FDA labeling, or lack of hematology involvement.

Missing molecular genetic confirmation of ADAMTS13 mutation
No documentation that the request is for on‑demand treatment of an acute TTP event
Dosing not consistent with FDA‑approved labeling for acute use
Prescriber is not a hematologist and no hematology consultation is documented

Definitions and Key Terms

inv-13: Adzynma (ADAMTS13, recombinant-krhn) — product identity and indication summary

Generic nameADAMTS13, recombinant-krhn

Brand nameAdzynma

Mechanism / classRecombinant human ADAMTS13 enzyme replacement (rADAMTS13) — plasma metalloprotease that cleaves VWF multimers

FDA‑approved indicationsProphylactic or on‑demand enzyme replacement therapy in adult and pediatric patients with congenital thrombotic thrombocytopenic purpura (cTTP)

Key study contextEvaluated in a global randomized, active‑controlled crossover study comparing ADAMTS13, recombinant-krhn to plasma‑based therapies in patients with cTTP

inv-14: Acute TTP event response criteria (trial definition) — resolution definitions used in studies

Trial resolution definition — platelet criterionPlatelet count ≥150,000/µL or platelet count within 25% of baseline (whichever occurs first)

Trial resolution definition — LDH criterionLDH ≤1.5× baseline or ≤1.5× upper limit of normal (ULN)

Additional requirement for responseResolution without requiring the use of another ADAMTS13‑containing agent

Efficacy endpoints usedProportion of acute TTP events responding to treatment and incidence of protocol‑defined acute and subacute TTP events

OpenPayer

Adzynma (ADAMTS13, Recombinant-Krhn) — Coverage Criteria for Congenital TTP

Coverage Criteria for Adzynma (ADAMTS13, recombinant-krhn)

Coding — HCPCS and Diagnosis Codes

Provider Actions, Authorization, and Documentation Requirements

Background

Definitions and Key Terms