CurrentViva Health PlanPolicy IC-0736

Adzynma coverage for congenital thrombotic thrombocytopenic purpura

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This policy governs prior authorization, dosing, renewal, billing, and coverage criteria for intravenous Adzynma (recombinant ADAMTS13) used to treat congenital thrombotic thrombocytopenic purpura, including prophylactic and on-demand therapy.

Policy Summary

PayerViva Health Plan

PolicyAdzynma coverage for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation of biallelic pathogenic ADAMTS13 variants and ADAMTS13 activity below 10% by the FRETS-VWF73 assay, unless the member is receiving prophylactic plasma infusion therapy.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2+Minimum member age

1800Maximum units per 28 days

6 monthsProphylaxis initial authorization

3 monthsOn-demand initial authorization

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CurrentViva Health PlanPolicy IC-0736

Adzynma coverage for congenital thrombotic thrombocytopenic purpura

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Get alerted when Policy IC-0736 changes without checking for updates manually.

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Policy Summary

PayerViva Health Plan

PolicyAdzynma coverage for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation of biallelic pathogenic ADAMTS13 variants and ADAMTS13 activity below 10% by the FRETS-VWF73 assay, unless the member is receiving prophylactic plasma infusion therapy.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2+Minimum member age

1800Maximum units per 28 days

6 monthsProphylaxis initial authorization

3 monthsOn-demand initial authorization

Acute attacks supported per 4 weeks

Coverage Criteria

Initial Therapy Criteria

Covered for initial therapy when all universal, diagnostic, and applicable treatment-use criteria are met.

Initial approval requirements

Universal and eligibility criteria
- Age: Member is at least 2 years of age.≥2 years
- Diagnostic exclusion: Member has not been diagnosed with another cTTP-like disorder, including acquired TTP, immune TTP, another primary thrombotic microangiopathy, immune thrombocytopenia, or Evans Syndrome.
- Inhibitor exclusion: Member does not have a medical history or presence of functional ADAMTS13 inhibitors before starting therapy.
- Contraindication screening: Member does not have an FDA-labeled contraindication to Adzynma, including a manifested life-threatening hypersensitivity reaction to Adzynma or its components.
- Monitoring: ADAMTS13 activity and ADAMTS13 neutralizing antibodies will be assessed before initiation and periodically during treatment.
  Applies to members previously exposed or naïve to plasma-based products.
Congenital TTP diagnosis: Member has documented severe hereditary ADAMTS13 deficiency confirmed by molecular genetic testing showing biallelic pathogenic ADAMTS13 variants, and ADAMTS13 activity below 10% by the FRETS-VWF73 assay.ADAMTS13 activity <10%
Members currently receiving prophylactic plasma infusion therapy may exceed 10% ADAMTS13 activity at therapy initiation.
Treatment use
- Prophylactic therapy: Treatment will be used as prophylactic therapy and the member has a history of at least one TTP event or is currently receiving prophylactic plasma infusion therapy.≥1 TTP event or current prophylactic plasma infusion therapy
  Members receiving prophylactic plasma-based therapies should discontinue routine use after achieving a therapeutic response.
- On-demand therapy: Treatment will be used as on-demand therapy and the member is at risk of a disease exacerbation.

Continuation Therapy Criteria

Covered for continuation therapy when all continued eligibility, safety, and response requirements are met.

Continuation therapy requirements

Continued eligibility: Member continues to meet the universal and applicable indication-specific criteria from the initial approval section.
Safety: Member has no unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions or neutralizing antibodies.
Response compared with pretreatment baseline
- Symptomatic improvement: If symptomatic, member has improvement in disease signs and symptoms, such as neurologic symptoms, renal dysfunction, TTP-related pain, or seizures.
- Therapy-specific response

Coverage is excluded when the member has another cTTP-like disorder, including acquired or immune TTP, another primary thrombotic microangiopathy, immune thrombocytopenia, or Evans Syndrome; has a history of or current functional ADAMTS13 inhibitors before therapy; or has an FDA-labeled contraindication to Adzynma, such as a manifested life-threatening hypersensitivity reaction to Adzynma or its components.

The preceding coverage information applies to non-Medicare coverage determinations. Medicare outpatient Part B coverage for drugs is governed by the Medicare Benefit Policy Manual, applicable National Coverage Determinations and Local Coverage Determinations, and any relevant Local Coverage Articles. Providers must comply with those Medicare policies where applicable; additional indications may be applied at the health plan’s discretion.

Billing and Clinical Coding

HCPCS CodeHCPCS

J7171

Injection, adamts13, recombinant-krhn, 10 iu; 1 billable unit = 10 units

NDCNDC

64764-0130-xx	Adzynma 500 IU single-dose vial for injection
64764-0135-xx	Adzynma 1500 IU single-dose vial for injection

Covered Diagnosis CodesICD-10Covered

D69.42

Congenital and hereditary thrombocytopenia purpura

Minimum Age

Minimum ageAt least 2 years of age

Applies toInitial Adzynma approval

Policy requirementMember must be at least 2 years old

ADAMTS13 Activity

Required activityLess than 10%

AssayFluorescent resonance energy transfer von Willebrand factor 73 (FRETS-VWF73) assay

Therapy-start exception

Authorization and Documentation Requirements

Prior Authorization

Initial Prior Authorization Required

Prior authorization is required for initial treatment. Approval requires documentation that the member meets the age, diagnosis, safety, laboratory, and treatment-use criteria in the policy, including the applicable prophylactic or on-demand pathway.

Member is at least 2 years of age.
Member does not have another congenital TTP-like disorder or alternate diagnosis, including acquired or immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, or Evans Syndrome.
Member has no history of, or current, functional ADAMTS13 inhibitors before therapy.
Member has no FDA-labeled contraindication to Adzynma.
Document the applicable cTTP confirmation, ADAMTS13 activity, treatment intent, and prophylactic or on-demand eligibility criteria.

Prior Authorization

Renewal Prior Authorization Required

Renewal authorization requires continued eligibility and evidence of benefit. The member must continue to meet applicable universal and indication-specific criteria, have no unacceptable toxicity, and demonstrate response compared with the pretreatment baseline.

Clinical Background

Congenital thrombotic thrombocytopenic purpura (cTTP) is a hereditary disorder caused by severe deficiency of the ADAMTS13 enzyme. It can produce thrombocytopenia, microangiopathic hemolytic anemia, and complications involving specific organs. Adzynma provides recombinant ADAMTS13 for prophylactic prevention or on-demand treatment of acute cTTP events. Coverage evaluation uses genetic confirmation, ADAMTS13 activity, neutralizing-antibody monitoring, and clinical or laboratory response to treatment.

Definitions and Diagnostic Terms

Severe Hereditary ADAMTS13 Deficiency

Confirmation methodMolecular genetic testing

Required findingBiallelic pathogenic variants in ADAMTS13

Condition definedSevere hereditary ADAMTS13 deficiency

ADAMTS13 Activity Criterion

Activity thresholdADAMTS13 activity below 10%

AssayFluorescent resonance energy transfer von Willebrand factor 73 (FRETS-VWF73) assay

Testing contextMembers receiving prophylactic plasma infusion therapy may exceed 10% activity at therapy initiation

Initial Therapy

Initial Therapy Criteria

Initial therapy requires all universal, diagnostic, and applicable treatment-use criteria.

Initial Adzynma therapy

Eligibility: Member is at least 2 years old, does not have another cTTP-like disorder, and has no functional ADAMTS13 inhibitors before treatment.Age ≥2 years
Universal criteria: Member has no FDA-labeled contraindication to Adzynma and will undergo ADAMTS13 activity and neutralizing antibody assessment before initiation and periodically during treatment.
Confirmed cTTP: Severe hereditary ADAMTS13 deficiency is confirmed by molecular genetic testing showing biallelic pathogenic ADAMTS13 variants and ADAMTS13 activity below 10% by the FRETS-VWF73 assay.

Renewal Criteria

Renewal requires continued initial criteria, no unacceptable toxicity, and documented response compared with pretreatment baseline.

Renewal requirements

Continued eligibility: Member continues to meet the universal and relevant indication-specific initial criteria.
Safety: Member has no unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions or neutralizing antibodies.
Response compared with pretreatment baseline
- Symptomatic response: If symptomatic, signs and symptoms improve, including neurologic symptoms, renal dysfunction, TTP-related pain, or seizures.

Quantity Limits

Quantity Limit

DrugAdzynma

Maximum quantity1,800 billable units

Time periodEvery 28 days

Policy Summary

PayerViva Health Plan

PolicyAdzynma coverage for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation of biallelic pathogenic ADAMTS13 variants and ADAMTS13 activity below 10% by the FRETS-VWF73 assay, unless the member is receiving prophylactic plasma infusion therapy.

SourceLink

On This Page

Prophylaxis response: Member has a reduction in or absence of an acute TTP event or a sub-acute TTP event.Acute event: platelet drop ≥50% of baseline or platelet count <100,000/μL and LDH >2× baseline or >2× ULN

A sub-acute event includes thrombocytopenia or microangiopathic hemolytic anemia with organ-specific signs or symptoms, including renal dysfunction, neurologic symptoms, fever, fatigue or lethargy, or abdominal pain.

On-demand response: Member has responded to an acute TTP event with improvement in thrombocytopenia or microangiopathic hemolytic anemia.Platelet drop ≥25% of baseline or platelet count <150,000/mcL; or LDH >1.5× baseline or >1.5× ULN

Members receiving prophylactic plasma infusion therapy may exceed 10% ADAMTS13 activity at therapy initiation

Acute TTP Event: Platelet Criterion

Baseline criterionPlatelet count drops by at least 50% from baseline

Absolute-count criterionPlatelet count below 100,000/μL

Event definitionEither platelet criterion is used with the required LDH elevation

Acute TTP Event: LDH Criterion

Baseline criterionLDH greater than 2 times baseline

ULN criterionLDH greater than 2 times the upper limit of normal (ULN)

Event definitionLDH elevation accompanies the qualifying platelet-count criterion

On-Demand Thrombocytopenia Response

Baseline platelet criterionPlatelet count drops by at least 25% from baseline

Absolute-count criterionPlatelet count below 150,000/mcL

UseDefines improvement in thrombocytopenia after on-demand treatment of an acute TTP event

On-Demand Hemolytic Anemia Response

Baseline criterionLDH greater than 1.5 times baseline

ULN criterionLDH greater than 1.5 times the upper limit of normal (ULN)

UseDefines improvement in microangiopathic hemolytic anemia after on-demand treatment

Document continued eligibility under the initial approval criteria.
Document absence of unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions and neutralizing antibodies.
Submit clinical and laboratory evidence of response for the applicable prophylactic or on-demand treatment pathway.
For on-demand treatment, account for the cumulative medication on hand and quantity needed to treat up to 4 acute attacks per 4 weeks during the authorization period, unless otherwise specified.

Prior Authorization

Prior Authorization Applies

Prior authorization applies to this drug under the plan’s utilization-management NQTL process. The assessment applies comparably, and not more stringently, to MH/SUD and medical/surgical Medical Benefit Prescription Drugs.

Indication was considered a factor supporting prior authorization.
Safety and efficacy were considered factors supporting prior authorization.
Potential for misuse or abuse was not considered a priority for prior authorization.
Drug cost was considered a factor supporting prior authorization.

Unsupported block type: documentation

Denial Risk

Age, Diagnosis, or Inhibitor Exclusion

Initial approval may be denied when basic eligibility requirements are not met. Document age, the confirmed diagnosis, and the absence of alternate disorders or pre-existing functional inhibitors.

Member is younger than 2 years of age.
The member has another congenital TTP-like disorder or alternate diagnosis, including acquired or immune TTP, another primary thrombotic microangiopathy, immune thrombocytopenia, or Evans Syndrome.
The member has a medical history of, or current, functional ADAMTS13 inhibitors before therapy.

Denial Risk

FDA-Labeled Contraindication

Approval may be denied when an FDA-labeled contraindication is present. Do not request or continue therapy when the member has a labeled contraindication, including a manifested life-threatening hypersensitivity reaction to Adzynma or its components.

Document review of FDA-labeled contraindications before initiation and renewal.
Life-threatening hypersensitivity to Adzynma or its components is a denial risk.

Denial Risk

Insufficient cTTP Confirmation

Approval may be denied when severe hereditary ADAMTS13 deficiency is not adequately confirmed. The record must support both the molecular diagnosis and the required functional activity criterion, including any documented exception related to current prophylactic plasma infusion.

No molecular genetic confirmation of biallelic pathogenic ADAMTS13 variants.
No documented ADAMTS13 activity below 10% by the FRETS-VWF73 assay, or no documentation explaining activity above 10% in a member currently receiving prophylactic plasma infusion therapy.
Incomplete or conflicting documentation of cTTP diagnosis may prevent approval.

Denial Risk

Failure of Renewal Criteria

Renewal may be denied when continued-coverage criteria are not met. The record must show ongoing eligibility, acceptable tolerability, and clinically meaningful response to treatment.

Unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions, is present.
Neutralizing antibodies or other evidence of treatment-related toxicity is present without acceptable management or supporting rationale.
The member does not demonstrate the required symptomatic, prophylactic, or on-demand response compared with pretreatment baseline.
Required renewal documentation or evidence of continued universal and indication-specific eligibility is missing.

Acute TTP Event

Platelet criterionPlatelet count drops by at least 50% from baseline or falls below 100,000/μL

LDH criterionLDH is greater than 2 times baseline or greater than 2 times ULN

DefinitionAn acute TTP event requires the qualifying platelet-count change together with the LDH elevation

Sub-Acute TTP Event

ThrombocytopeniaA thrombocytopenia event

Hemolytic anemiaA microangiopathic hemolytic anemia event

Organ-specific manifestationsMay include renal dysfunction, neurologic symptoms, fever, fatigue or lethargy, and abdominal pain

On-Demand Response

Thrombocytopenia responsePlatelet count drops by at least 25% from baseline or falls below 150,000/mcL

Hemolytic anemia responseLDH is greater than 1.5 times baseline or greater than 1.5 times ULN

Treatment contextResponse follows treatment of an acute TTP event with on-demand therapy

D69.42

ICD-10-CM codeD69.42

DiagnosisCongenital and hereditary thrombocytopenia purpura

Coverage statusCovered diagnosis code

ADAMTS13 activity <10%

Members currently receiving prophylactic plasma infusion therapy may exceed 10% ADAMTS13 activity at therapy initiation.

Prophylaxis or on-demand use

Prophylaxis: Prophylactic therapy is planned and the member has at least one TTP event or is currently receiving prophylactic plasma infusion therapy.
On-demand therapy is planned and the member is at risk of disease exacerbation.

Prophylaxis or on-demand response

Prophylaxis: Member has a reduction in or absence of an acute or sub-acute TTP event.Acute event: platelet drop ≥50% of baseline or platelet count <100,000/μL and LDH >2× baseline or >2× ULN
A sub-acute event includes thrombocytopenia or microangiopathic hemolytic anemia with organ-specific signs and symptoms.
On-demand: Member has responded to an acute TTP event with improvement in thrombocytopenia or microangiopathic hemolytic anemia.Platelet drop ≥25% of baseline or platelet count <150,000/mcL; or LDH >1.5× baseline or >1.5× ULN