XOLREMDI (mavorixafor)

XOLREMDI (mavorixafor)

Self-Administration-oral capsules

Diagnosis considered for coverage:

WHIM syndrome: Indicated in patients 12 years of age and older with WHIM syndrome (warts, hypogammaglobulinemia, infections and myelokathexis) to increase the number of circulating mature neutrophils and lymphocytes.

Coverage Criteria:

For diagnosis of WHIM syndrome:

• Diagnosis of WHIM (warts, hypogammaglobulinemia, infections and myelokathexis) syndrome; AND

• Patient has genotype confirmed variant of CXCR4 as detected by an FDA-approved test or a test performed at a facility approved by Clinical Laboratory Improvement Amendments (CLIA); AND

• Patient has an absolute neutrophil count (ANC) less than or equal to 500 cells /µL; AND 

• Patient is 12 years of age or older; AND

• Prescribed by or in consultation with one of the following: 

  • Immunologist 

  • Hematologist 

  • Geneticist 

  • Allergist 

Reauthorization Criteria:

For diagnosis of WHIM syndrome:

• Patient demonstrates positive clinical response to therapy (e.g., improvement in ANC, reduction in infections)

Coverage Duration:

• Initial: 6 months

• Reauthorization: 1 year

Dosing:

For diagnosis of WHIM syndrome:

• Dosing is based on body weight:

  • Weight >50 kg: 400 mg once daily 

  • Weight ≤50 kg: 300 mg once daily

  • Dosage may be further adjusted based on multiple drug-drug interactions

• Medication should be taken on an empty stomach after an overnight fast, and at least 30 minutes before food

• Reduce daily dosage to 200 mg when used concomitantly with strong CYP3A4 inhibitors

Authorization is not covered for the following:

• The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information: 

• XOLREMDI, a selective CXCR4 antagonist, is the first therapy specifically indicated in patients with WHIM syndrome.

• Use of XOLREMDI is contraindicated with drugs that are highly dependent on CYP2D6 for clearance.

• Obtain an electrocardiogram when initiating, during concomitant use, and as clinically indicated in patients receiving concomitant medications with a known potential to prolong the QTc interval.

• Per consult with immunologist/allergist, severe neutropenia (less than or equal to 500 cells per µL) is used in standard practice as a cut off for ANC levels.

 Policy Updates:

• 12/01/2024 – New policy for Xolremdi approved by WHA P&T Committee. (P&T, 11/20/2024)

References:

1. Xolremdi Prescribing Information. X4 Pharmaceuticals, Inc. Boston, MA. June 2024. 

2. Per clinical consult with immunologist/ allergist, June 27, 2024.