CurrentEmblemHealthPolicy MG.MM.PH.382

Lamzede (velmanase alfa-tycv) infusion for alpha‑mannosidosis

Covers clinical criteria, dosing, coding, and authorization requirements for Lamzede (velmanase alfa-tycv) for treatment of non‑CNS manifestations of alpha‑mannosidosis for EmblemHealth members.

Policy Summary

PayerEmblemHealth

PolicyLamzede (velmanase alfa-tycv) infusion for alpha‑mannosidosis

Policy CodePolicy MG.MM.PH.382

Change TypeCriteria addeddosing and coding updates

Effective DateApr 21, 2023

Next Review DateMar 24, 2025

Key ActionObtain prior authorization for 12 months with documentation supporting diagnosis and required clinical evidence, including diagnostic confirmation and genetic testing.

SourceLink

POLICY UPDATE CHANGES

Added requirement for biallelic pathogenic variants in MAN2B1 confirmed by mutation testing to Initial Criteria.

Updated dosing limits and added renewal criteria.

Updated J-code to J0217 and removed J3590.

Updated description of the current ICD-10 code; no criteria changes.

12 monthsLength of authorization

1 mg/kg weeklyStandard dosing

110 mgMax units/dose

J0217HCPCS/J-code

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Coverage Criteria for Lamzede (velmanase alfa-tycv)

Initial Therapy — approval when ALL of the following are met

Approve when ALL of the following are met:

Initial Criteria: 1) Confirmed diagnosis of alpha-mannosidosis defined as alpha-mannosidase activity less than 10% of normal activity in blood leukocytes; AND 2) Biallelic pathogenic variants in MAN2B1 (Mannosidase Alpha Class 2B Member 1) confirmed by mutation testing; AND 3) Presence of non-central nervous system manifestations (examples include progressive motor function disturbances, hearing and speech impairment, skeletal abnormalities, and immune deficiency); AND 4) Medication prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder subspecialist, or a physician who specializes in the treatment of lysosomal storage disorders.

Renewal Criteria — approval for continuation when ALL of the following are met

Approve renewal when ALL of the following are met:

Renewal Criteria: 1) Patient continues to meet Initial Criteria; AND 2) Absence of unacceptable toxicity (examples include anaphylaxis or severe allergic or infusion-associated reactions); AND 3) Patient has demonstrated a beneficial response to therapy or stabilization of disease.

This therapy is not indicated for central nervous system (CNS) manifestations of alpha‑mannosidosis. The policy limits the approved use to patients with non‑CNS manifestations, such as progressive motor disturbances, hearing and speech impairment, skeletal abnormalities, and immune deficiency. Requests for treatment of CNS disease do not meet the coverage indication described in this policy.

Use of this medication for central nervous system manifestations is outside the stated indication and is not supported by the policy. Approvals require documented presence of non‑central nervous system manifestations of alpha‑mannosidosis as specified in the initial criteria; CNS‑only presentations do not satisfy the policy’s coverage requirements.

Billing and Diagnosis Codes

Applicable HCPCS/J-codeHCPCSCovered

J0217

Injection, velmanase alfa-tycV, 1 mg

Applicable NDCsNDC

10122-0180-XX

Lamzede (velmanase alfa-tycv) 10mg

Relevant ICD-10 DiagnosesICD-10

E77.1

Defects in glycoprotein degradation

inv-08: Maximum dose/unit — dosing limits and frequency

Maximum per-dose limit110 mg every 7 days (HCPCS units)

Standard dosing frequency1 mg/kg once weekly (IV)

Infusion rate guidance (≤49 kg)Infuse over a minimum of 60 minutes once weekly

Infusion rate guidance (≥50 kg)Infuse at a maximum rate of 25 mL/hour once weekly

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Authorization will be provided for 12 months and may be renewed. Requests must include documentation supporting the diagnosis and that the patient meets the policy criteria.

Authorization period: 12 months
Required documentation: clinical evidence supporting diagnosis and policy criteria (e.g., enzymatic activity <10% and/or biallelic pathogenic MAN2B1 variants)
Prescriber requirement: prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder subspecialist, or lysosomal storage disorder specialist

Step Therapy

No step therapy is specified for this medication in this policy.

Background on Lamzede (velmanase alfa-tycv)

Lamzede (velmanase alfa‑tycv) is an enzyme replacement therapy indicated for the treatment of non‑central nervous system manifestations of alpha‑mannosidosis. The policy requires a confirmed diagnosis — defined by alpha‑mannosidase activity < 10% of normal in blood leukocytes — and genetic confirmation of biallelic pathogenic variants in MAN2B1. Indicated non‑CNS features include progressive motor function disturbances, hearing and speech impairment, skeletal abnormalities, and immune deficiency. Therapy must be prescribed by or in consultation with an appropriate specialist (for example, a geneticist, endocrinologist, metabolic disorder subspecialist, or physician experienced in lysosomal storage disorders).

Definitions and Clinical Manifestations

inv-14: Non-central nervous system manifestations

DefinitionClinical features of alpha‑mannosidosis outside the central nervous system (non‑CNS manifestations)

Examples — auditory and speechHearing loss and speech impairment (hearing and speech impairment)

Examples — motor and skeletalProgressive motor function disturbances and skeletal abnormalities

Examples — immuneImmune deficiency

Prescriber requirementMedication must be prescribed by or in consultation with a geneticist, endocrinologist, metabolic disorder subspecialist, or physician specializing in lysosomal storage disorders

Documentation Required

Required Clinical Documentation

The treating physician or primary care provider must submit clinical evidence demonstrating the member meets the policy criteria. Required evidence includes diagnostic confirmation (enzymatic testing showing alpha‑mannosidase activity <10% of normal and/or genetic testing demonstrating biallelic pathogenic MAN2B1 variants) and documentation of relevant clinical findings (for example non‑CNS manifestations, hearing/speech impairment, skeletal abnormalities, immune deficiency, progressive motor dysfunction). Failure to submit required documentation will prevent proper review and may result in denial or delay.

Diagnostic confirmation: enzymatic testing and/or genetic testing results
Clinical findings: documentation of non‑CNS manifestations and other relevant signs/symptoms
Prescriber/consult requirement: documentation that medication is prescribed by or in consultation with an appropriate specialist