CurrentCentenePolicy CP.PHAR.679

Mavorixafor (Xolremdi) — Coverage Criteria for WHIM Syndrome

Defines medical necessity and prior authorization criteria for mavorixafor (Xolremdi) for members age ≥12 with genetically confirmed WHIM syndrome, including initial and continued therapy requirements, dosing limits, and documentation expectations for Centene lines of business.

Policy Summary

PayerCentene

PolicyMavorixafor (Xolremdi) — Coverage Criteria for WHIM Syndrome

Policy CodePolicy CP.PHAR.679

Change TypeNo material change

Effective DateJul 1, 2024

Next Review Date

Key ActionSubmit prior authorization with genetic confirmation of a CXCR4 variant, specialist involvement, baseline ANC/ALC labs, infection history, current weight, and dosing within weight-based caps.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥12minimum approved age

ANC ≤400baseline neutropenia threshold for initiation

Max 400 mgmaximum daily dose

6 moinitial approval length

12 morenewal approval length

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Coverage Criteria for Mavorixafor (Xolremdi)

Initial Therapy — Covered when ALL of the following are met:

Covered when ALL of the following are met:

Initial WHIM criteria: 1) Diagnosis of WHIM syndrome confirmed by genetic confirmation of a CXCR4 variant; 2) Prescribed by or in consultation with a geneticist, hematologist, immunologist, or infectious disease specialist; 3) Age ≥ 12 years; 4) Baseline absolute neutrophil count (ANC) ≤ 400 cells/µL; 5) Documentation of baseline absolute lymphocyte count (ALC) and number of infections experienced within the last year; 6) Xolremdi is not prescribed concurrently with plerixafor (Mozobil); 7) Documentation of member's current weight (in kg); 8) Dose does not exceed weight-based limits: a) Weight > 50 kg: 400 mg/day (4 capsules); b) Weight ≤ 50 kg: 300 mg/day (3 capsules). Approval duration: 6 months

All conditions required

Continuation Therapy — Covered when ALL of the following are met:

Covered when ALL of the following are met:

Continuation WHIM criteria: 1) Member meets one of the following: a) Currently receiving medication via Centene benefit or previously met initial approval criteria; OR b) Currently receiving medication and enrolled in a state/product with continuity of care regulations; 2) Member is responding positively to therapy as evidenced by both: a) At least two instances of an ANC ≥ 500 cells/µL on two separate days within the last 6 months; and b) Either i) at least two instances of an ALC ≥ 1,000 cells/µL on two separate days within the last 6 months, OR ii) reduction from baseline in infections; 3) Xolremdi is not prescribed concurrently with plerixafor (Mozobil); 4) Documentation of member's current weight (in kg); 5) If request is for a dose increase, new dose does not exceed weight-based limits: a) Weight > 50 kg: 400 mg/day (4 capsules); b) Weight ≤ 50 kg: 300 mg/day (3 capsules). Approval duration: 12 months

All conditions required

Other Indications — Requests for uses not specified in initial/continued criteria:

Requests for uses not specified in initial/continued criteria:

Other indications: 1) If the drug has undergone a label change within the last 6 months not yet reflected in this policy, refer to applicable formulary/no-coverage or non-formulary policies for the relevant line of business; OR 2) If the requested use is not listed under section III (Diagnoses/Indications for which coverage is NOT authorized) and criterion 1 does not apply, refer to the off-label use policy for the relevant line of business (e.g., CP.CPA.09 for commercial, HIM.PA.154 for marketplace, CP.PMN.53 for Medicaid).

One of the two conditions suffices

Non‑FDA approved indications that are not specifically addressed in this policy are not authorized unless there is adequate documentation of efficacy and safety in accordance with the applicable off‑label use policy for the member’s line of business (see CP.CPA.09 for Commercial, HIM.PA.154 for Health Insurance Marketplace, and CP.PMN.53 for Medicaid) or other evidence of coverage documents.

Concomitant use of mavorixafor with medications that are highly dependent on CYP2D6 for clearance is a listed contraindication and such combinations are not permitted.

Use of mavorixafor for non‑FDA approved indications that are not addressed in this policy is not authorized when there is insufficient documentation of efficacy and safety; requests for off‑label use should follow the referenced off‑label use policies for the applicable line of business.

Product Codes, Dosing, and Lab Thresholds

Product availability / NDCNDC

NDC not listed

Product availability: Capsule 100 mg (no NDC provided in policy)

Weight-based dosing thresholds

Dosing threshold for members > 50 kg400 mg per day (4 capsules)

Dosing threshold for members ≤ 50 kg300 mg per day (3 capsules)

Maximum daily dose400 mg/day

Baseline ANC for initiation

Baseline ANC required for initiation≤ 400 cells/µL

ContextRequired as part of initial approval criteria for WHIM syndrome

Documentation

Prior Authorization, Documentation, and Provider Responsibilities

Prior Authorization

Prior Authorization Required

Prior authorization is required. Requests must demonstrate that all initial or continued therapy criteria for WHIM syndrome are met, including genetic confirmation of a CXCR4 variant, specialist involvement (geneticist, hematologist, immunologist, or infectious disease specialist), age and baseline lab thresholds, and required supporting documentation. Provider must submit office notes, lab results (ANC, ALC), documentation of number of infections in the prior year, and current weight in kg.

Initial therapy criteria include baseline ANC ≤ 400 cells/µL, age ≥ 12 years, genetic confirmation of CXCR4 variant, specialist prescribing/consultation, baseline ALC and infection history, and weight and dosing limits.
Continuation therapy requires evidence of prior approval or continuity of care and positive response: ≥2 ANC ≥ 500 cells/µL on separate days in last 6 months and either ≥2 ALC ≥ 1,000 cells/µL on separate days in last 6 months or reduction in infections.
Approval durations: Initial = 6 months; Continued = 12 months.

Denial Risk

Clinical Background

WHIM syndrome is a congenital immunodeficiency associated with pathogenic variants in CXCR4 and is characterized by warts, hypogammaglobulinemia, recurrent infections, and myelokathexis. Mavorixafor (Xolremdi) is an oral CXCR4 antagonist indicated to increase circulating mature neutrophils and lymphocytes in patients with WHIM syndrome and is the therapy addressed by this policy.

Terms and Abbreviations

ANC

ANCANC: absolute neutrophil count

UsageUsed as a laboratory measure in initial and continued therapy criteria

Abbreviation sourceDefined in Appendix A: Abbreviation/Acronym Key

ALC

ALCALC: absolute lymphocyte count

UsageUsed as a laboratory measure in continued therapy response criteria

Abbreviation sourceDefined in Appendix A: Abbreviation/Acronym Key

Policy Summary

PayerCentene

PolicyMavorixafor (Xolremdi) — Coverage Criteria for WHIM Syndrome

Policy CodePolicy CP.PHAR.679

Change TypeNo material change

Effective DateJul 1, 2024

Next Review Date

SourceLink

On This Page

Provider must submit baseline ANC with prior authorization request

Continued therapy ANC response

ANC response criteria for continuationAt least two instances of ANC ≥ 500 cells/µL on two separate days within the last 6 months

Timing windowWithin the most recent 6-month period

Evidence typeLaboratory results showing ANC on two separate days

Continued therapy ALC response

ALC response criteria for continuationAt least two instances of ALC ≥ 1,000 cells/µL on two separate days within the last 6 months

Alternative response evidenceOr reduction from baseline in infections (documented) as evidence of clinical response

DocumentationSubmit ALC lab results or infection history as part of continuation review

Requests will be denied if documentation is incomplete or missing required elements. Common denial triggers include absence of genetic confirmation of a CXCR4 variant, missing baseline ANC (or ANC > 400 cells/µL for initial therapy), lack of documentation of baseline ALC or infection history, missing current weight, or failure to show prior approval/continuity and clinical response for continuation requests.

No genetic confirmation of a CXCR4 variant — denial risk.
For initial therapy, baseline ANC > 400 cells/µL or missing ANC — denial risk.
Incomplete supporting documentation (office notes, labs, infection count, weight) — denial risk.

Step Therapy

Step Therapy / Concomitant Therapy

No step therapy is specified. Concomitant use with plerixafor (Mozobil®) is prohibited for both initial and continued therapy.

Concomitant therapy: Xolremdi must not be prescribed concurrently with plerixafor (Mozobil®).
Step therapy: none specified.

CXCR4

CXCR4 definitionCXCR4: CXC chemokine receptor 4

RelevanceTherapeutic target of mavorixafor (Xolremdi) and required genetic confirmation of a CXCR4 variant for diagnosis

SourceDescribed in drug description and Appendix A