CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0736

Adzynma (recombinant ADAMTS13) for Congenital Thrombotic Thrombocytopenic Purpura

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Prior authorization, dosing, coverage criteria, renewal, and billing for intravenous Adzynma in members with severe hereditary ADAMTS13 deficiency and congenital thrombotic thrombocytopenic purpura, including prophylactic and on-demand therapy.

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyAdzynma for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewApr 1, 2026

Next Review DateN/A

Key ActionSubmit molecular testing confirming biallelic pathogenic ADAMTS13 variants and activity below 10% by the FRETS-VWF73 assay.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

Age 2+Minimum age

<10%ADAMTS13 threshold

1,800Maximum units per 28 days

6 monthsProphylaxis initial authorization

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CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0736

Adzynma (recombinant ADAMTS13) for Congenital Thrombotic Thrombocytopenic Purpura

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Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyAdzynma for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewApr 1, 2026

Next Review DateN/A

Key ActionSubmit molecular testing confirming biallelic pathogenic ADAMTS13 variants and activity below 10% by the FRETS-VWF73 assay.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

Age 2+Minimum age

<10%ADAMTS13 threshold

1,800Maximum units per 28 days

6 monthsProphylaxis initial authorization

On-demand initial authorization

1Covered diagnosis codes

Medical Necessity and Coverage Criteria

Initial approval for cTTP

Covered when all universal and cTTP criteria are met.

Initial approval

Universal criteria
- Age: Member is at least 2 years of age.Age ≥2 years
- Alternate diagnoses excluded: Member has not been diagnosed with another congenital TTP-like disorder, including acquired or immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, or Evans Syndrome.
- ADAMTS13 inhibitors absent: Member does not have a medical history or presence of functional ADAMTS13 inhibitors before the start of therapy.
- Contraindications absent: Member does not have an FDA-labeled contraindication to Adzynma.
Severe hereditary ADAMTS13 deficiency
- Genetic confirmation: Molecular genetic testing confirms biallelic pathogenic variants in ADAMTS13.
- ADAMTS13 activity: Member has ADAMTS13 activity below 10% as measured by the fluorescent resonance energy transfer von Willebrand factor 73 (FRETS-VWF73) assay.ADAMTS13 activity <10%
  Members currently receiving prophylactic plasma infusion therapy may exceed 10% ADAMTS13 activity at the start of therapy.
Treatment pathway
- Prophylactic therapy
  - Treatment approach: Treatment will be used as prophylactic therapy.
  - Prior TTP history or current plasma prophylaxis: Member has a history of at least one TTP event or is currently receiving prophylactic plasma infusion therapy.At least 1 prior 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 approach and exacerbation risk: Treatment will be used as on-demand therapy and the member is at risk of a disease exacerbation.

Renewal therapy

Renewal requires all continuing eligibility, safety, and response criteria.

Renewal criteria

Continued eligibility: Member continues to meet the universal and other relevant indication-specific criteria from initial approval.
Safety: There is an absence of unacceptable toxicity from the drug.
Examples of unacceptable toxicity include severe hypersensitivity or anaphylactic reactions.
Clinical response
- Symptomatic improvement: If symptomatic, signs and symptoms of disease have improved compared with the pretreatment baseline, including neurologic symptoms, renal dysfunction, TTP-related pain, or related manifestations.

Coverage under this policy is limited to members with congenital thrombotic thrombocytopenic purpura (cTTP). Other cTTP-like or alternate thrombotic microangiopathy disorders are excluded, including acquired or immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, and Evans Syndrome. Requests are also excluded when the member has a history of, or currently has, functional ADAMTS13 inhibitors before therapy or an FDA-labeled contraindication to Adzynma.

Billing and Coding Requirements

Covered HCPCS CodesHCPCSCovered

J7171

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

Covered NDC CodesNDCCovered

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

Covered ICD-10 Diagnosis CodesICD-10Covered

D69.42

Congenital and hereditary thrombocytopenia purpura

Minimum Age

Minimum member ageAt least 2 years of age

Applies toInitial approval criteria

Below thresholdMembers younger than 2 years do not meet the initial approval age criterion

ADAMTS13 Activity

Required activityADAMTS13 activity below 10%

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

Therapy exception

Authorization, Documentation, and Denial Rules

Prior Authorization

Prior Authorization and Authorization Periods

Prior authorization is required. Initial and renewal authorization periods depend on whether Adzynma is used for prophylaxis or on-demand therapy. For on-demand therapy, authorization must account for the member’s cumulative medication on hand and support treatment of up to 4 acute attacks per 4 weeks during the authorization period.

Prophylaxis: initial authorization for 6 months (180 days); renewal every 12 months (365 days).
On-demand: initial authorization for 3 months (90 days); renewal every 3 months (90 days).
On-demand authorization provides sufficient cumulative quantity to treat up to 4 acute attacks per 4 weeks for the authorization duration.

Prior Authorization

Prior Authorization Applies

Utilization management and prior authorization apply comparably, and not more stringently, to mental health/substance use disorder and medical/surgical medical-benefit prescription drugs. The policy’s prior authorization factors include indication and drug cost; safety and efficacy and potential misuse/abuse were not identified as PA priorities.

Clinical Background

Congenital thrombotic thrombocytopenic purpura (cTTP) is a hereditary thrombotic microangiopathy caused by severe ADAMTS13 deficiency. Adzynma replaces recombinant ADAMTS13 and may be used as prophylactic therapy to reduce TTP events or as on-demand therapy for acute events. Coverage requires molecular confirmation of severe hereditary ADAMTS13 deficiency and clinical documentation supporting the selected treatment approach.

Policy Definitions

Severe Hereditary ADAMTS13 Deficiency

Molecular findingBiallelic pathogenic variants in ADAMTS13 confirmed by molecular genetic testing

Laboratory findingADAMTS13 activity below 10%

Required assayFRETS-VWF73 assay

Acute TTP Event

Platelet findingPlatelet count drop of at least 50% from baseline or platelet count below 100,000/μL

LDH findingLDH greater than 2 times baseline or greater than 2 times ULN

DefinitionAn acute TTP event includes the platelet finding together with the LDH elevation

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyAdzynma for congenital thrombotic thrombocytopenic purpura

Policy CodePolicy IC-0736

Change TypeNo material change

Effective DateN/A

Last ReviewApr 1, 2026

Next Review DateN/A

Key ActionSubmit molecular testing confirming biallelic pathogenic ADAMTS13 variants and activity below 10% by the FRETS-VWF73 assay.

SourceLink

On This Page

Prophylaxis response: There is a reduction in or absence of an acute TTP event or a subacute TTP event.Acute event: platelet count drop ≥50% of baseline or platelet count <100,000/μL together with LDH >2× baseline or >2× ULN

Subacute events include thrombocytopenia or microangiopathic hemolytic anemia with organ-specific signs or symptoms, including renal dysfunction, neurologic symptoms, fever, fatigue or lethargy, and/or abdominal pain.

On-demand response: An acute TTP event responds to therapy, demonstrated by improvement in thrombocytopenia or microangiopathic hemolytic anemia.Platelet drop ≥25% of baseline or platelet count <150,000/mcg/L, or LDH >1.5× baseline or >1.5× ULN

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

Dosing Limit

Maximum billable units1,800 billable units

Time periodEvery 28 days

Unit basisHCPCS units

Acute TTP Platelet Criterion

Platelet-count criterionPlatelet count drop of at least 50% from baseline

Alternative platelet criterionPlatelet count below 100,000/μL

Event contextUsed as part of the acute TTP event definition

Acute TTP LDH Criterion

LDH criterionLDH greater than 2 times baseline

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

Event contextUsed together with the platelet criterion to define an acute TTP event

On-Demand Platelet Response

Platelet response criterionPlatelet decrease of at least 25% from baseline

Alternative platelet criterionPlatelet count below 150,000/mcg/L

Response contextIndicates improvement in thrombocytopenia after on-demand therapy

On-Demand LDH Response

LDH response criterionLDH greater than 1.5 times baseline

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

Response contextIndicates improvement in microangiopathic hemolytic anemia after on-demand therapy

Applies to the requested medical-benefit prescription drug or drug group.
Indication: considered for prior authorization.
Cost of drug: considered for prior authorization.
Safety and efficacy: prior authorization not a priority.
Potential for misuse/abuse: prior authorization not a priority.

Documentation Required

Diagnostic and Indication Documentation

Submit documentation establishing severe hereditary ADAMTS13 deficiency and the clinical indication for Adzynma in congenital thrombotic thrombocytopenic purpura (cTTP). Documentation must support the applicable prophylactic or on-demand treatment pathway.

Molecular genetic testing confirms biallelic pathogenic variants in ADAMTS13.
ADAMTS13 activity is <10% by the FRETS-VWF73 assay; members receiving prophylactic plasma infusion therapy may exceed 10% at treatment start.
For prophylaxis, document a history of at least one TTP event or current prophylactic plasma infusion therapy.
For on-demand therapy, document that the member is at risk of disease exacerbation.
Members receiving prophylactic plasma-based therapy should discontinue routine use after achieving a therapeutic response.

Documentation Required

Renewal Response Documentation

For renewal, document that the member continues to meet applicable universal and indication-specific criteria, has no unacceptable toxicity, and has responded to therapy compared with the pre-treatment baseline.

Document continuing eligibility under the initial approval criteria.
Document absence of unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions.
For symptomatic disease, document improvement in signs and symptoms such as neurologic symptoms, renal dysfunction, or TTP-related pain.
For prophylaxis, document reduction or absence of acute or sub-acute TTP events and associated organ-specific signs and symptoms.
For on-demand treatment, document response to the acute TTP event through improvement in thrombocytopenia or microangiopathic hemolytic anemia.

Documentation Required

Weight-Based Dose Documentation

Document the member’s current body weight and calculate the administration dose and volume using the actual recombinant ADAMTS13 potency printed on each vial, not the nominal potency.

Prophylaxis: 40 IU/kg once every other week; frequency may be adjusted to 40 IU/kg once weekly based on prior prophylactic dosing or clinical response.
On-demand: 40 IU/kg on treatment day 1, 20 IU/kg on day 2, and 15 IU/kg once daily from day 3 onward until two days after the acute event resolves.
Record the weight-based calculated dose and administration volume using the vial’s actual potency in IU.

Denial Risk

Ineligible Member or Alternate Diagnosis

Coverage may be denied when the member is not eligible under the initial approval criteria or has an alternate thrombotic or hematologic diagnosis that does not meet the policy’s cTTP requirements.

Member must be at least 2 years of age.
Deny for other congenital TTP-like or alternate disorders, including acquired or immune TTP, other primary thrombotic microangiopathies, immune thrombocytopenia, or Evans Syndrome.
Deny when there is a medical history or presence of functional ADAMTS13 inhibitors before therapy begins.

Denial Risk

FDA-Labeled Contraindication

Do not approve Adzynma when the member has an FDA-labeled contraindication to the requested agent.

Confirm that the member does not have any FDA-labeled contraindication before initial approval or renewal.

Denial Risk

cTTP Criteria Not Met

Coverage requires confirmation of severe hereditary ADAMTS13 deficiency and the applicable cTTP treatment indication. Deny when the required diagnostic or clinical criteria are not documented or are not met.

Deny if biallelic pathogenic ADAMTS13 variants are not confirmed by molecular genetic testing.
Deny if ADAMTS13 activity is not <10% by the FRETS-VWF73 assay, subject to the policy exception for members currently receiving prophylactic plasma infusion therapy at treatment initiation.
Deny if prophylactic therapy is not supported by a prior TTP event or current prophylactic plasma infusion therapy.
Deny if on-demand therapy is not supported by risk of disease exacerbation.

Denial Risk

Renewal Response or Safety Failure

Deny or do not renew authorization when the member no longer meets applicable eligibility criteria, has unacceptable toxicity, or lacks documented clinical response compared with baseline.

Deny renewal if universal or indication-specific criteria are no longer met.
Deny renewal for unacceptable toxicity, including severe hypersensitivity or anaphylactic reactions.
Deny renewal if symptomatic disease has not improved.
For prophylaxis, deny renewal without reduction or absence of acute or sub-acute TTP events and related organ-specific manifestations.
For on-demand therapy, deny renewal without documented improvement in thrombocytopenia or microangiopathic hemolytic anemia after an acute TTP event.

Billing Rule

Quantity and Dosing Limit Exceeded

Authorization and billing are subject to the policy’s quantity and dosing limits. Excess units or quantities beyond operational need may be denied or limited, including medication held for on-demand acute attacks.

Maximum: 1,800 HCPCS billable units every 28 days.
Consider the member’s cumulative medication on hand when authorizing on-demand therapy.
On-demand quantity is limited to the amount sufficient to maintain medication on hand for up to 4 acute attacks per 4 weeks during the authorization period, unless otherwise specified.
Deny or limit units that exceed the applicable dose, authorization period, or operational quantity requirement.

Subacute TTP Event

Hematologic eventA thrombocytopenia event or a microangiopathic hemolytic anemia event

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

Event typeSubacute TTP event

On-Demand Treatment Response

Platelet improvementPlatelet decrease of at least 25% from baseline or platelet count below 150,000/mcg/L

LDH improvementLDH greater than 1.5 times baseline or greater than 1.5 times ULN

Treatment contextResponse to on-demand therapy following an acute TTP event

Congenital Thrombotic Thrombocytopenic Purpura (cTTP)

Preferred termCongenital thrombotic thrombocytopenic purpura

AbbreviationcTTP

Synonymous condition namesUpshaw-Schulman syndrome; hereditary thrombotic thrombocytopenic purpura