CurrentAmeriHealth Caritas VIP CarePolicy MA08.171

ADAMTS13, recombinant-krhn (Adzynma)

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This policy governs medical-necessity coverage and utilization requirements for ADAMTS13, recombinant-krhn (Adzynma) in adult and pediatric Medicare Advantage members with congenital thrombotic thrombocytopenic purpura (cTTP). It covers prophylactic therapy and on-demand treatment of acute TTP events when the stated diagnostic, specialist-prescribing, and dosing criteria are met.

Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyADAMTS13, recombinant-krhn (Adzynma)

Policy CodePolicy MA08.171

Change TypeAdministrative reissueno material clinical or coverage change

Effective DateDec 15, 2025

Next Review DateN/A

Key ActionSubmit dose, frequency, or regimen changes to the Company for a new approval.

SourceLink

POLICY UPDATE CHANGES

The policy was reissued in accordance with the Company's annual review process on September 18, 2026.

OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.

CurrentAmeriHealth Caritas VIP CarePolicy MA08.171

ADAMTS13, recombinant-krhn (Adzynma)

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Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyADAMTS13, recombinant-krhn (Adzynma)

Policy CodePolicy MA08.171

Change TypeAdministrative reissueno material clinical or coverage change

Effective DateDec 15, 2025

Next Review DateN/A

Key ActionSubmit dose, frequency, or regimen changes to the Company for a new approval.

SourceLink

POLICY UPDATE CHANGES

The policy was reissued in accordance with the Company's annual review process on September 18, 2026.

Covered treatment pathways

40 IU/kgStandard prophylactic dose

40/20/15 IU/kgOn-demand dosing sequence

<10%Required ADAMTS13 activity at diagnosis

Not coveredOther uses

Covered Uses and General Coverage Rules

Initial prophylactic therapy

Covered when all criteria are met.

Diagnosis of cTTP with biallelic ADAMTS13 mutations confirmed by molecular genetic testing and ADAMTS13 activity below 10% at the time of diagnosis.ADAMTS13 activity <10%
Treatment purpose: Adzynma is prescribed for routine prophylactic treatment to prevent TTP events.
Specialist involvement: Prescribed by or in consultation with a hematologist.
Dose and frequency: 40 IU/kg body weight intravenously once every other week; the prophylaxis dosing frequency may be adjusted to 40 IU/kg body weight once weekly based on the prior prophylactic dosing regimen or clinical response.40 IU/kg IV every other week; may adjust to once weekly

Continuation prophylactic therapy

Covered when all criteria are met.

Prior treatment: The individual previously received routine prophylactic treatment with Adzynma.
Positive clinical response: Documentation shows a positive clinical response to Adzynma therapy.
Examples include reduction or maintenance of TTP events, increased platelet count, or decreased lactate dehydrogenase (LDH) level.
Specialist involvement: Prescribed by or in consultation with a hematologist.

On-demand treatment of acute TTP events

Covered when all criteria are met.

On-demand treatment of an acute TTP event

Diagnosis of cTTP with biallelic ADAMTS13 mutations confirmed by molecular genetic testing and ADAMTS13 activity below 10% at the time of diagnosis.ADAMTS13 activity <10%
Treatment purpose: Adzynma is prescribed for on-demand treatment of an acute TTP event.
Specialist involvement: Prescribed by or in consultation with a hematologist.
Dose and frequency: Administer 40 IU/kg body weight on day 1, 20 IU/kg body weight on day 2, and 15 IU/kg body weight on day 3 and beyond until 2 days after the acute event is resolved.

Adzynma is covered for prophylactic treatment of congenital thrombotic thrombocytopenic purpura (cTTP) and for on-demand treatment of an acute TTP event when the policy criteria are met. All other uses are considered experimental/investigational and are not covered unless supported as an accepted off-label use under the Company's policy for off-label coverage of prescription drugs and biologics.

Applicable Billing and Diagnosis Codes

CPT Procedure Code Number(s)CPT

N/A

ICD-10 Procedure Code Number(s)ICD-10

N/A

ICD-10 Diagnosis Code Number(s)ICD-10

D69.42

Congenital and hereditary thrombocytopenia purpura

HCPCS Level II Code Number(s)HCPCS

J7171

Injection, adamts13, recombinant-krhn, 10 iu

Revenue Code Number(s)Revenue

N/A

ADAMTS13 Activity Threshold

Required ADAMTS13 activity at diagnosisLess than 10%

Applies toIndividuals diagnosed with congenital thrombotic thrombocytopenic purpura (cTTP)

Required confirmationADAMTS13 mutations confirmed by molecular genetic testing with biallelic mutations in the ADAMTS13 gene

Authorization, Documentation, and Denial Risks

Prior Authorization

New Approval Required for Regimen Changes

Submit a new approval request whenever the member’s Adzynma dose, frequency, or regimen changes, including changes related to weight or incomplete therapeutic response.

A new approval is required for any dose, frequency, or regimen change.
Requests outside the policy dosing and frequency requirements require supporting published peer-reviewed literature.

Documentation Required

Medical-Record Documentation Required

Ensure the medical record documents the medical necessity for Adzynma treatment. Records must be available to the Company upon request, and failure to provide them may result in denial.

Acceptable sources may include professional provider office records, hospital records, nursing home records, home health agency records, therapy records, and test reports.

Clinical Background

Congenital thrombotic thrombocytopenic purpura (cTTP) is an autosomal recessive disorder caused by biallelic mutations in the ADAMTS13 gene. These mutations result in severe ADAMTS13 enzyme deficiency, generally reflected by ADAMTS13 activity of less than 10%, allowing ultra-large von Willebrand factor multimers to accumulate and contribute to abnormal small-vessel clotting.

Key Terms

ADAMTS13, recombinant-krhn (Adzynma)

Therapy typeHuman recombinant ADAMTS13 enzyme replacement therapy

Prophylactic indicationProphylactic treatment to prevent TTP events in adult and pediatric individuals with congenital thrombotic thrombocytopenic purpura (cTTP)

On-demand indicationOn-demand treatment of an acute TTP event in adult and pediatric individuals with cTTP

Congenital Thrombotic Thrombocytopenic Purpura (cTTP)

DisorderAn autosomal recessive form of thrombotic thrombocytopenic purpura

Genetic basisCaused by biallelic mutations in the ADAMTS13 gene

Initial Treatment Pathways

Initial prophylactic therapy

Initial prophylactic therapy is covered when all criteria are met.

Initial prophylaxis

Diagnosis: ADAMTS13 mutations are confirmed by molecular genetic testing with biallelic mutations in the ADAMTS13 gene, and ADAMTS13 activity is less than 10% at the time of diagnosis.ADAMTS13 activity <10%
Treatment purpose: Adzynma is prescribed for routine prophylactic treatment to prevent TTP events.
Specialist involvement: Adzynma is prescribed by or in consultation with a hematologist.

Continuation Therapy

Continuation prophylaxis

Continuation is covered when all criteria are met.

Continuation criteria

Prior treatment: The individual has previously received routine prophylactic treatment with Adzynma.
Positive clinical response: Documentation shows a positive clinical response to Adzynma therapy.
Examples include reduction or maintenance of TTP events, increased platelet count, or decreased lactate dehydrogenase (LDH) level.
Specialist involvement: Adzynma is prescribed by or in consultation with a hematologist.

Policy Summary

PayerAmeriHealth Caritas VIP Care

PolicyADAMTS13, recombinant-krhn (Adzynma)

Policy CodePolicy MA08.171

Change TypeAdministrative reissueno material clinical or coverage change

Effective DateDec 15, 2025

Next Review DateN/A

Key ActionSubmit dose, frequency, or regimen changes to the Company for a new approval.

SourceLink

On This Page

40 IU/kg day 1; 20 IU/kg day 2; 15 IU/kg day 3 onward

Continue until 2 days after the acute event is resolved.

The Company may review and audit services regardless of provider participation status.

Documentation Required

Clinical Authorization Documentation

Authorization requests must include clinical documentation supporting the diagnosis and treatment criteria for cTTP, including genetic confirmation and ADAMTS13 activity results.

Document a diagnosis of cTTP.
Provide molecular genetic testing confirming biallelic mutations in the ADAMTS13 gene.
Document an ADAMTS13 activity level below 10% at the time of diagnosis.
Identify the treatment as routine prophylaxis to prevent TTP events or on-demand treatment of an acute TTP event, as applicable.
Confirm that Adzynma is prescribed by or in consultation with a hematologist.

Documentation Required

Continuation Response Documentation

For continuation requests, document that the individual previously received routine prophylactic Adzynma and has demonstrated a positive clinical response to therapy.

Positive response may include reduction or maintenance of TTP events, increased platelet count, or decreased lactate dehydrogenase (LDH) level.
Adzynma must continue to be prescribed by or in consultation with a hematologist.

Prior Authorization

Exception-Dose Support Required

Requests for Adzynma dosing outside the policy requirements must include supporting published peer-reviewed literature submitted by the prescribing professional provider.

Routine prophylaxis: 40 IU/kg intravenously once every other week, with adjustment to once weekly when supported by the prior prophylactic regimen or clinical response.
Acute on-demand treatment: 40 IU/kg on day 1, 20 IU/kg on day 2, and 15 IU/kg on day 3 and beyond until 2 days after the acute event resolves.
Use Company-recognized pharmacology compendia and current published evidence when supporting an exception dose.

Denial Risk

Noncovered Indications

Adzynma is not covered for uses outside covered routine prophylactic treatment to prevent TTP events or on-demand treatment of an acute TTP event, unless an applicable accepted off-label coverage policy supports the indication.

All other uses are considered experimental/investigational and are not covered unless supported as an accepted off-label use under the Company’s policy for prescription drugs and biologics.

Denial Risk

Failure to Meet Clinical Criteria

Coverage may be denied when required clinical criteria are not met or cannot be documented, including cTTP diagnosis, biallelic ADAMTS13 mutations, ADAMTS13 activity below 10%, hematology involvement, covered treatment purpose, or policy-conforming dosing.

Denial risk applies when molecular genetic testing does not confirm biallelic ADAMTS13 mutations.
Denial risk applies when ADAMTS13 activity is not documented as below 10% at diagnosis.
Denial risk applies when the prescriber is not a hematologist and there is no hematologist consultation.
Denial risk applies when prophylactic or acute on-demand treatment criteria and dosing requirements are not met.

Denial Risk

Unsupported Dosing or Missing Records

Flag requests that lack required medical-record documentation or supporting literature for dosing outside the policy requirements. The Company may deny the service when requested information is not produced.

Medical records must substantiate medical necessity and be available upon request.
Requests outside the listed dosing and frequency requirements must include published peer-reviewed literature supporting the requested regimen.
The Company reserves the right to conduct postpayment review and audit procedures for Adzynma claims.

Characteristic ADAMTS13 activity

Less than 10% of normal

Associated mechanismADAMTS13 deficiency causes accumulation of ultra-large von Willebrand factor multimers, leading to abnormal clotting in small blood vessels

Dose and frequency: Adzynma is administered at 40 IU/kg body weight intravenously once every other week. The prophylaxis dosing frequency may be adjusted to 40 IU/kg body weight once weekly based on the prior prophylactic dosing regimen or clinical response.40 IU/kg IV every other week; may adjust to once weekly

Initial on-demand treatment

Initial on-demand therapy is covered when all criteria are met.

Acute-event treatment

Diagnosis: ADAMTS13 mutations are confirmed by molecular genetic testing with biallelic mutations in the ADAMTS13 gene, and ADAMTS13 activity is less than 10% at the time of diagnosis.ADAMTS13 activity <10%
Treatment purpose: Adzynma is prescribed for on-demand treatment of an acute TTP event.
Specialist involvement: Adzynma is prescribed by or in consultation with a hematologist.
Dose and frequency: Administer 40 IU/kg body weight on day 1, 20 IU/kg body weight on day 2, and 15 IU/kg body weight on day 3 and beyond until 2 days after the acute event is resolved.40 IU/kg day 1; 20 IU/kg day 2; 15 IU/kg day 3 onward
Continue until 2 days after the acute event is resolved.