CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Lamzede (velmanase alfa-tycv) (Intravenous) — Coverage Criteria

Clinical coverage criteria for Lamzede (velmanase alfa-tycv) intravenous therapy for treatment of non‑CNS manifestations of alpha‑mannosidosis for Neighborhood Health Plan of Rhode Island members (Medicaid, Commercial, Medicare).

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyLamzede (velmanase alfa-tycv) (Intravenous) — Coverage Criteria

Policy CodePolicy N/A

Change TypeMinor — no material clinical or coverage changes

Effective Date05/01/2024

Next Review DateN/A

Key ActionSubmit prior authorization documenting age (3–35 years), definitive diagnosis (enzyme activity <10% and biallelic MAN2B1 pathogenic variants), baseline labs/functional measures, and specialist prescribing or consultation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial authorization length

110 mg weeklymax dosing

J0217HCPCS

E77.1ICD-10

3–35 yrsage range

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Coverage Criteria for Lamzede (velmanase alfa-tycv)

inv-01: Initial Approval

Covered when ALL of the following are met:

Age: Patient is between 3 and 35 years of age3-35 years

Prescriber: Medication prescribed by or in consultation with a specialist familiar with the treatment of lysosomal storage disorders

Baseline measurements: Documented baseline serum oligosaccharides

Baseline functional measures: Documented baseline age-appropriate values for one or more functional measures (e.g., 6-minute walk test, 3-minute stair climb test, pulmonary function tests such as forced vital capacity, motor function/BOT-2). For very young patients in which FVC or 6-MWT are not suitable, requests will be reviewed on a case-by-case basis.

Definitive diagnosis: Identification of deficient acid alpha-mannosidase enzyme activity in peripheral blood leukocytes or other nucleated cells (e.g., fibroblasts) of <10% of normal activity AND identification of biallelic pathogenic variants in MAN2B1 by molecular genetic testingenzyme activity <10%

Clinical phenotype: Patient has signs and symptoms consistent with mild or moderate alpha-mannosidosis (e.g., absence of neurological manifestations, able to ambulate independently)able to ambulate independently

Targeted manifestations: Therapy intended to treat non-central nervous system manifestations of alpha-mannosidosis (e.g., myopathy, motor function disturbances, immunodeficiency)non-CNS manifestations only

Reproductive safety: If patient is a female of reproductive potential, confirmed negative pregnancy test

inv-02: Renewal

Renewal permitted when ALL of the following are met:

Continued eligibility: Patient continues to meet the initial criteria

Tolerability: Absence of unacceptable toxicity from the drug (examples include anaphylaxis, severe allergic or infusion-associated reactions)

Clinical benefit or stabilization: Patient has demonstrated benefit or stabilization compared to pretreatment age-appropriate baseline in one or more measures: stability or improvement in serum oligosaccharide concentration, 6-MWT, 3-MSCT, or FVC (% predicted), or stabilization/slowing of disease progression/clinical decline

Coverage will not be provided for members who meet any of the following exclusion criteria: the patient cannot walk without support; history of hematopoietic stem cell transplant (HSCT) or bone marrow transplant; presence of a known chromosomal abnormality or syndrome affecting psychomotor development other than alpha‑mannosidosis; or total IgE level of >800 IU/ml. These exclusions are part of the initial approval criteria and must be documented with the prior authorization request.

inv-17: Molecular confirmation

Molecular confirmation (required): Identification of biallelic pathogenic variants in MAN2B1 by molecular genetic testing

This molecular confirmation is required in addition to enzyme assay demonstrating <10% activity for definitive diagnosis and coverage

There are no separate top‑level eligibility nodes beyond the specific coverage criteria listed elsewhere in this policy. Providers should follow the detailed initial approval and renewal requirements for patient selection and documentation.

Billing and Diagnosis Codes

Covered HCPCS CodesHCPCSCovered

J0217

Injection, velmanase alfa-tycv, 1 mg; 1 billable unit = 1 mg

NDCNDC

10122-0180-xx

Lamzede 10 mg lyophilized powder NDC

Covered Diagnosis Codes (ICD-10)ICD-10Covered

E77.1

Defects in glycoprotein degradation

inv-07: Enzyme activity threshold

Enzyme activity threshold<10% of normal activity (acid alpha-mannosidase in peripheral blood leukocytes or other nucleated cells)

Specimen types referencedPeripheral blood leukocytes or other nucleated cells (e.g., fibroblasts)

Role in coverageRequired as part of definitive diagnosis for initial approval of therapy

inv-08: Total IgE exclusion threshold

Total IgE exclusion threshold>800 IU/ml

Effect on coverageTotal IgE >800 IU/ml is an explicit exclusion for coverage

Provider Responsibilities and Documentation Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial approval is for 6 months. Documentation submitted with the prior authorization request must include the patient’s age and required baseline tests described below.

Initial approval length: 6 months
Renewal: may be renewed for additional 6-month periods

Step Therapy

Medicare Prior Therapy Exception

Medicare members who have received this medication within the past 365 days are exempt from step therapy requirements.

Documentation Required

Covered Indication and Diagnostic Requirements

inv-17: Covered indication criteria

Covered indication criteria — molecular confirmation required alongside enzyme assay showing <10% activity:

Enzyme assay: Identification of deficient acid alpha-mannosidase enzyme activity in peripheral blood leukocytes or other nucleated cells (e.g., fibroblasts) of <10% of normal activity<10% of normal activity

Molecular confirmation: Identification of biallelic pathogenic variants in MAN2B1 by molecular genetic testing

Both enzyme assay (<10%) AND biallelic MAN2B1 pathogenic variants are required for coverage

Key Definitions

inv-16: Definitive diagnosis definition

Definitive diagnosis componentsRequires BOTH identification of deficient acid alpha-mannosidase enzyme activity (<10% of normal) AND identification of biallelic pathogenic variants in MAN2B1 by molecular genetic testing.

PurposeEstablishes molecular and biochemical confirmation of alpha-mannosidosis prior to coverage

Where measuredEnzyme activity measured in peripheral blood leukocytes or other nucleated cells (e.g., fibroblasts); genetic testing by sequencing for MAN2B1 variants.

Background and Clinical Context

Alpha‑mannosidosis is an ultra‑rare lysosomal storage disorder caused by pathogenic variants in MAN2B1, resulting in deficient alpha‑mannosidase activity and accumulation of oligosaccharides. Clinical features include recurrent infections, hearing impairment, cognitive and motor deficits, and other systemic manifestations. Definitive diagnosis requires both identification of <10% of normal acid alpha‑mannosidase activity in peripheral blood leukocytes or other nucleated cells and molecular confirmation of biallelic pathogenic variants in MAN2B1. Lamzede (velmanase alfa‑tycv) is indicated to treat non‑central nervous system (non‑CNS) manifestations of alpha‑mannosidosis.

Non-Covered Uses

Use of Lamzede (velmanase alfa‑tycv) at doses or for conditions outside the FDA‑recognized indications, or for uses not supported by standard compendia or the peer‑reviewed literature, is considered investigational and is not covered by Neighborhood Health Plan of Rhode Island.

Policy Revision History

06/04/2025reviewedLatest

Policy reviewed (Dates Reviewed: 02/12/2024, 06/04/2025) — most recent review recorded.

05/01/2024effective

Policy effective date established for Lamzede (velmanase alfa-tycv): 05/01/2024.

02/12/2024reviewed

Policy review documented on 02/12/2024 (prior to effective date).