CurrentCommunity-CarePolicy N/A

Adzynma

Defines medical-benefit coverage criteria for initial and continued use of rADAMTS13 (Adzynma) to treat congenital thrombotic thrombocytopenic purpura (cTTP), quantity limits, authorization period, and applicable billing code(s).

Policy Summary

PayerCommunity-Care

PolicyAdzynma

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionObtain molecular genetic testing confirming cTTP and laboratory documentation of ADAMTS13 activity < 10 IU/dL and absence of anti-ADAMTS13 autoantibodies; prescribe or consult with a hematologist or rare genetic hematologic disease specialist.

SourceLink

POLICY UPDATE CHANGES

No material clinical/coverage changes

1Primary Indication covered

1Applicable HCPCS code

Coverage Summary

Coverage stance: covered_with_criteria for Adzynma (rADAMTS13) to treat congenital thrombotic thrombocytopenic purpura (cTTP). Coverage requires genetic confirmation of cTTP and biochemical evidence of severe ADAMTS13 deficiency without anti-ADAMTS13 autoantibodies (ADAMTS13 activity < 10 IU/dL).

ADAMTS13 activity threshold< 10 IU/dL (< 10% of normal) at time of diagnosis

Authorization period1 year (if approved; subject to benefit changes)

Specialist requirementPrescribed by or in consultation with a hematologist or specialist in rare genetic hematologic diseases

Diagnostic documentationMolecular genetic testing confirming cTTP and absence of anti-ADAMTS13 autoantibodies required

Weight requirementMember weight required for approval (used to determine dosing/quantity)

Initial Therapy Criteria

Initial Approval

Covered when ALL of the following are met:

Initial Approval - cTTP

Specialist prescriber: Prescribed by, or in consultation with, a hematologist or specialist in rare genetic hematologic diseases
Molecular genetic confirmation: Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP) confirmed by molecular genetic testing
ADAMTS13 activity threshold: ADAMTS13 activity < 10% of normal (< 10 IU/dL) at the time of diagnosis< 10 IU/dL
No autoantibodies: Anti-ADAMTS13 autoantibodies are NOT present

Prior Authorization

Specialist involvement required

Prescription must be by, or in consultation with, a hematologist or specialist in rare genetic hematologic diseases for initial approval.

Documentation Required

Diagnostic confirmation required

Molecular genetic testing confirming cTTP and laboratory documentation of ADAMTS13 activity < 10 IU/dL and absence of anti-ADAMTS13 autoantibodies must be provided for initial approval.

Billing Rule

Weight required for approval

A member weight is required for approval (used to determine dosing/quantity). Applicable billing code: J7171.

J7171

Continuation Therapy Criteria

Continuation of Therapy

Covered when ALL of the following are met:

Continuation of Therapy - cTTP

Diagnosis of congenital thrombotic thrombocytopenic purpura (cTTP)
Clinical response: Member has achieved a positive clinical response (e.g. improvement in acute and subacute TTP events, TTP manifestations, and clinical symptoms) and is appropriate to continue therapy with rADAMTS13 (Adzynma)

Documentation Required

Clinical response documentation for continuation

For continuation of therapy, documentation that the member has achieved a positive clinical response (e.g., improvement in acute and subacute TTP events, TTP manifestations, and clinical symptoms) and is appropriate to continue rADAMTS13 is required.

Prior Authorization

Authorization period

If approved, coverage is allowed for 1 year; prior authorization must be renewed thereafter.

Provider Actions & Documentation Requirements

Provider actions: prior authorization required with prescription by (or consultation with) a hematologist or specialist in rare genetic hematologic diseases; submit molecular genetic testing confirming cTTP and laboratory documentation showing ADAMTS13 activity < 10 IU/dL and absence of anti-ADAMTS13 autoantibodies; include member weight for dosing/quantity (HCPCS J7171 used for billing); prior authorization is granted for up to 1 year and must be renewed for continuation with documentation of positive clinical response.

Actionable requirements for approval

Required documentation

Unsupported block type: list

Background & Definitions

Adzynma (recombinant ADAMTS13, rADAMTS13) is indicated for treatment of congenital thrombotic thrombocytopenic purpura (cTTP). Initial coverage requires confirmation of cTTP by molecular genetic testing and laboratory evidence of severe ADAMTS13 deficiency with ADAMTS13 activity < 10 IU/dL at diagnosis, and demonstration that anti-ADAMTS13 autoantibodies are not present.

Term	Definition
cTTP	Congenital thrombotic thrombocytopenic purpura due to inherited ADAMTS13 deficiency.