CurrentBlue Cross Blue Shield - IowaPolicy 05.05.45

Xolremdi (mavorixafor) for WHIM syndrome

Defines benefit application, prior authorization requirements, coverage criteria, dosing limits, and documentation needed for Xolremdi (mavorixafor) in members with WHIM syndrome for Blue Cross Blue Shield - Iowa.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyXolremdi (mavorixafor) for WHIM syndrome

Policy CodePolicy 05.05.45

Change TypeNo material change

Effective DateAug 22, 2024

Next Review Date

Key ActionObtain prior authorization with documentation of CXCR4 genotype–confirmed WHIM syndrome, baseline ANC (<400 cells/µL), and compatible clinical history.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 moinitial authorization

12 mocontinuation authorization

120/30dquantity limit

12+minimum age

Coverage Criteria for Xolremdi (mavorixafor)

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Initial authorization criteria: Diagnosis of WHIM syndrome confirmed by genotype variant of CXCR4

Authorization of 6 months may be granted for members 12 years of age and older when all criteria are met.

Baseline laboratory criterion: Baseline absolute neutrophil count (ANC) < 400 cells/µL< 400 cells/µL

Clinical history consistent with WHIM syndrome (e.g., repeated bacterial infections, persistent warts)

Continuation Therapy

Covered when ANY of the following demonstrate response for continuation authorization:

Continuation response criteria: Reduced infection frequency relative to pretreatment

Continuation response criteria: Increased ANC and/or ALC relative to baseline

Continuation response criteria: Reduction of warts on the skin and genital warts

Benefit determinations are based on the applicable contract language in effect at the time services are rendered. Exclusions, limitations, or exceptions may apply, benefits may vary by contract, and individual member benefits must be verified. This policy may not apply to Federal Employee Program (FEP) members; Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable.

Billing and Coding

Provider billing codes (not specified)mixed

No codes listed

Baseline absolute neutrophil count (ANC)

Baseline ANC threshold< 400 cells/µL

Applies toInitial authorization for members ≥12 years

Role in criteriaRequired along with CXCR4 genotype confirmation and consistent clinical history

Quantity limit

Standard quantity limit120 capsules per 30 days

Product strengthXolremdi (mavorixafor) 100 mg capsule

FDA recommended dosing (for reference)Weight >50 kg: 400 mg orally once daily; Weight <50 kg: 300 mg orally once daily

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for initiation and continuation of mavorixafor (Xolremdi) for WHIM syndrome. Initial approvals may be granted for up to 6 months for members 12 years and older; continuation approvals require documentation of ongoing benefit and may be subject to approval renewal.

Submit prior authorization request before initiating therapy
Initial approval duration: up to 6 months for eligible members ≥12 years
Continuation requests require documentation of ongoing clinical benefit

Documentation Required

Supportive Therapy Context

Document prior supportive therapies and prior use of off‑label plerixafor where applicable. Supportive care may include colony stimulating factors, immunoglobulin replacement, and appropriate anti‑infective therapies. Evidence of prior or concurrent use of these therapies and the member's response should be included in the request when relevant to clinical decision making.

List supportive therapies tried (e.g., CSFs, IVIG, prophylactic antibiotics) and response
If plerixafor was used off‑label, provide dates, dosing, and clinical outcome

Documentation Required

Required Documentation

Provide the following documentation with initial prior authorization requests and with continuation/renewal requests as applicable. Incomplete submissions may delay review or result in denial.

Medical records confirming diagnosis of WHIM syndrome with CXCR4 pathogenic variant (molecular genetic test report)
Baseline laboratory results: absolute neutrophil count (ANC) and absolute lymphocyte count (ALC); baseline ANC <400 cells/µL required for initial approval
Clinical history documenting recurrent infections, persistent warts, hypogammaglobulinemia, or other WHIM‑related manifestations
For continuation: recent chart notes and lab reports showing clinical improvement or disease stability on therapy
If available, bone marrow biopsy results showing myelokathexis and prior supportive therapy use (CSFs, IVIG, anti‑infectives, off‑label plerixafor) including response

Denial Risk

Non‑conforming Requests

Requests that do not meet the documented clinical criteria, lack required documentation, or request mavorixafor for indications other than WHIM syndrome as defined in policy will be considered not medically necessary and may be denied.

Requests without CXCR4 genetic confirmation (unless documentation explains why testing was not possible) may be denied
Requests lacking baseline ANC/ALC or clinical history consistent with WHIM may be denied
Use for other diagnoses or experimental/uncertain indications will be considered not medically necessary

Background

WHIM syndrome (Warts, Hypogammaglobulinemia, Infections, and Myelokathexis) is an ultra‑rare combined primary immunodeficiency most commonly caused by pathogenic variants in the CXCR4 gene. It is characterized by neutropenia, lymphopenia, hypogammaglobulinemia, recurrent infections, warts, and myelokathexis. Symptoms typically begin in early childhood and can range from mild to life‑threatening; diagnosis relies on compatible clinical features, complete blood count with differential (showing neutropenia/lymphopenia), bone marrow findings, and confirmation by molecular genetic testing for CXCR4 variants.

Definitions

WHIM syndrome

WHIM acronymWarts, hypogammaglobulinemia, infections, and myelokathexis

Pathogenesis noteMost cases due to CXCR4 receptor gene variants causing impaired mobilization of neutrophils and lymphocytes

Clinical featuresRecurrent viral/bacterial infections, skin and genital warts, neutropenia and variable lymphopenia

Diagnostic approachDiagnosis based on symptoms, CBC with differential, bone marrow findings (myelokathexis), and molecular genetic testing