Currentharvard pilgrim health carePolicy N/A

Adzynma (ADAMTS13, recombinant-krhn) prior authorization for congenital TTP

Prior authorization guideline for Adzynma (ADAMTS13, recombinant-krhn) for prophylactic or on‑demand enzyme replacement therapy in adult and pediatric patients with congenital thrombotic thrombocytopenic purpura (cTTP); applies to specified Harvard Pilgrim/Tufts plan products including Medicare Advantage and Tufts Health One Care.

Policy Summary

Payerharvard pilgrim health care

PolicyAdzynma (ADAMTS13, recombinant-krhn) prior authorization for congenital TTP

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateApr 1, 2024

Next Review DateN/A

Key ActionObtain prior authorization and submit molecular genetic testing plus ADAMTS13 activity (<10%) and specialist documentation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

ADAMTS13 <10%Diagnostic activity threshold required

6 monthsInitial approval duration

12 monthsReapproval duration

J7171PA required code

Coverage Criteria for Adzynma (recombinant ADAMTS13)

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inv-01: Initial Therapy

Covered when ALL of the following are met:

Initial Authorization criteria: 1) Documented diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP) confirmed by BOTH: (a) molecular genetic testing; AND (b) ADAMTS13 activity <10% measured by the FRET‑VWF73 assay. 2) Prescribed by or in consultation with a hematologist, oncologist, or specialist in rare genetic hematologic diseases. 3) If the request is for prophylactic therapy, documentation of ONE of the following: (a) history of ≥1 documented TTP event; OR (b) currently receiving prophylactic Adzynma to manage cTTP.ADAMTS13 activity <10% (FRET‑VWF73)

Initial authorizations provided for 6 months.

inv-02: Reauthorization / Continuation Therapy

Reauthorization when ALL of the following are met:

Reauthorization criteria: 1) Ongoing documented diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP) confirmed by BOTH: (a) molecular genetic testing; AND (b) ADAMTS13 activity <10% measured by the FRET‑VWF73 assay. 2) Prescribed by or in consultation with a hematologist, oncologist, or specialist in rare genetic hematologic diseases. 3) Documentation of therapeutic response as evidenced by ONE of the following: (a) reduction/improvement in acute TTP events defined as ≥50% drop from baseline in platelet count, or platelet count <100,000/µL AND LDH elevation >2x baseline or >2x ULN; OR (b) reduction/improvement in subacute TTP events defined as thrombocytopenia or microangiopathic hemolytic anemia with organ-specific signs/symptoms (e.g., renal dysfunction, neurologic symptoms, fever, fatigue, abdominal pain); OR (c) reduction/improvement in TTP manifestations defined as ≥25% drop in platelets from baseline, or platelet count <150,000/µL, or LDH >1.5x baseline or >1.5x ULN.ADAMTS13 activity <10% (FRET‑VWF73)

Reauthorizations provided for 12 months; members new to the plan stable on Adzynma should be reviewed against initial criteria.

Use of Adzynma is limited to patients with congenital thrombotic thrombocytopenic purpura (cTTP) as defined by the FDA‑approved indication. This guideline does not cover immune‑mediated (acquired) TTP or other non‑congenital causes of severe ADAMTS13 deficiency. Authorization is intended for enzyme replacement therapy (prophylactic or on‑demand) in individuals with documented hereditary ADAMTS13 deficiency due to ADAMTS13 gene mutations and clinical features of cTTP, consistent with the product labeling.

Requests that do not include required diagnostic confirmation or adequate specialty documentation may be denied. Specifically, submitters must provide molecular genetic testing confirming cTTP and objective laboratory evidence of severe ADAMTS13 deficiency (ADAMTS13 activity <10% by FRET‑VWF73 assay), and the request should be prescribed by or in consultation with a hematologist, oncologist, or specialist in rare genetic hematologic diseases. For reauthorization, include documentation of therapeutic response per the criteria in the policy (for example reductions in acute or subacute TTP events or improvements in platelet counts and LDH). Missing or inadequate documentation of these elements may render the request not medically necessary under this guideline.

Provider Actions, Documentation, and Billing

Prior Authorization

Prior Authorization Required

Prior authorization is required for HCPCS J7171 (INJECTION ADAMTS13 RECOMBINANT-KRHN 10 IU). Requests without prior authorization may be denied.

HCPCS J7171 — INJECTION ADAMTS13 RECOMBINANT-KRHN 10 IU

Note

Action required by the ordering provider: follow payer prior authorization and documentation workflows. If an unlabeled provider action is present in source materials, treat it as an alert to verify authorization and documentation before submitting the request.

Documentation Required

Required Documentation

Documentation must include molecular genetic testing confirming congenital thrombotic thrombocytopenic purpura (cTTP) and ADAMTS13 activity < 10%. Also include prescribing or consultation details from a hematologist, oncologist, or specialist in rare genetic hematologic diseases.

Molecular genetic testing confirming cTTP
ADAMTS13 activity < 10% (measured by FRET-VWF73 assay)
Prescribed by or in consultation with a hematologist, oncologist, or specialist in rare genetic hematologic diseases

Denial Risk

Diagnostic Documentation Required

Diagnostic documentation is required. Requests that lack molecular genetic testing confirming cTTP or ADAMTS13 activity < 10% may be denied. Ensure lab reports and genetic test results are submitted with the authorization request.

Missing molecular genetic testing or ADAMTS13 < 10% may result in denial
Submit lab reports (ADAMTS13 activity) and genetic testing results with request

Definitions

cTTP — definition

DefinitionCongenital thrombotic thrombocytopenic purpura (cTTP) — hereditary ADAMTS13 deficiency caused by mutations in the ADAMTS13 gene leading to severe enzyme deficiency and thrombotic microangiopathy

Clinical featuresCharacterized by life‑threatening acute crises and multiple chronic symptoms; high untreated mortality for acute events

Treatment contextIndication for Adzynma (rADAMTS13) is prophylactic or on‑demand enzyme replacement therapy in adults and children with cTTP

ADAMTS13 activity threshold — definition

Threshold definitionADAMTS13 activity < 10% as measured by the fluorescent resonance energy transfer (FRET) - von Willebrand factor 73 (VWF73) assay

Role in authorizationRequired alongside molecular genetic testing to confirm severe ADAMTS13 deficiency for initial authorization and reauthorization

Source study requirementClinical trials and approval populations required molecular confirmation and ADAMTS13 activity <10% by FRET‑VWF73

OpenPayer

Adzynma (ADAMTS13, recombinant-krhn) prior authorization for congenital TTP

Coverage Criteria for Adzynma (recombinant ADAMTS13)

Coding and Diagnostic Thresholds

Provider Actions, Documentation, and Billing

Background

Definitions