CurrentCignaPolicy IP0654

Complement System Disorders - WHIM Syndrome - Xolremdi

Defines Cigna coverage and prior authorization criteria for Xolremdi (mavorixafor capsules) for treatment of WHIM syndrome in patients ≥12 years, including initial and continuation approval conditions, duration, prescribing specialist requirements, and non-covered uses.

Policy Summary

PayerCigna

PolicyComplement System Disorders - WHIM Syndrome - Xolremdi

Policy CodePolicy IP0654

Change TypeNew (09/15/2024); Annual revision (08/01/2025)

Effective DateAug 1, 2025

Next Review Date

Key ActionPrior Authorization is recommended; initial approval requires prescription by or consultation with an immunologist, hematologist, or dermatologist and is granted for 1 year.

SourceLink

POLICY UPDATE CHANGES

New policy created.

Annual revision performed.

1Covered Indication(s)

1 yearApproval Duration

≥12Minimum Age (years)

Coverage Summary

Defines Cigna coverage and prior authorization criteria for Xolremdi (mavorixafor capsules) for treatment of WHIM syndrome in patients ≥ 12 years, including initial and continuation approval conditions, duration, prescribing specialist requirements, and non-covered uses. Coverage is covered_with_criteria and approvals are provided for 1 year.

Prior Authorization & Documentation Requirements

Prior Authorization

Prior authorization required

Prior Authorization is recommended for benefit coverage of Xolremdi; approvals provided for the duration noted (1 year) and initial approval requires prescription by or consultation with a specialist (immunologist, hematologist, or dermatologist).

Approval duration: 1 year

Documentation Required

Confirmatory genetic testing required for initial therapy

Genetic testing confirming pathogenic or likely pathogenic CXCR4 variant must be documented for initial therapy.

Documentation Required

Baseline laboratory values required for initial therapy

Baseline absolute neutrophil count or white blood cell count demonstrating ≤ 400 cells/µL must be documented.

Documentation Required

Continuation benefit documentation

For current users, prescriber must document continuing benefit via recent objective measurement (examples: reduced infection frequency/duration/severity, less antibiotic use, fewer warts, or improved/stabilized clinical signs/lab counts).

Billing Rule

Approval duration

Approvals are for 1 year.

Clinical Evidence & References

Evidence includes the FDA prescribing information: Xolremdi oral capsules prescribing information, X4 Pharmaceuticals; April 2024, and a phase 3 trial report: Results of a phase 3 trial of mavorixafor for WHIM syndrome (presented 2023). Clinical evidence for Xolremdi in WHIM is limited to rare-disease trial data and the available prescribing information.

Background & Definitions

Xolremdi (mavorixafor) is a CXCR4 antagonist indicated to increase circulating mature neutrophils and lymphocytes for treatment of WHIM syndrome, a rare autosomal primary immunodeficiency often due to heterozygous CXCR4 mutations that causes chronic neutropenia and recurrent infections.

Term	Definition
WHIM syndrome	A rare autosomal primary immunodeficiency characterized by warts, hypogammaglobulinemia, infections and myelokathexis due to CXCR4 receptor hyperactivity.

Policy Summary

PayerCigna

PolicyComplement System Disorders - WHIM Syndrome - Xolremdi

Policy CodePolicy IP0654

Change TypeNew (09/15/2024); Annual revision (08/01/2025)

Effective DateAug 1, 2025

Next Review Date

Key ActionPrior Authorization is recommended; initial approval requires prescription by or consultation with an immunologist, hematologist, or dermatologist and is granted for 1 year.

SourceLink

On This Page

OpenPayer

Complement System Disorders - WHIM Syndrome - Xolremdi

Coverage Summary

Initial and Continuation Therapy Criteria (WHIM syndrome)

Prior Authorization & Documentation Requirements

Applicable Codes

Clinical Evidence & References

Background & Definitions

Revision History