Mavorixafor

XOLREMDI is 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 lympocytes. XOLREMDI is a CXC chemokine receptor 4 antagonist 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.

Mavorixafor is an orally bioavailable CXCR4 antagonist that blocks the binding of the CXCR4 ligand, stromal-derived factor-1α (SDF-1α)/CXC Chemokine Ligand 12 (CXCL12). SDF-1/CXCR4 plays a role in trafficking and homing of leukocytes to and from the bone marrow compartment. Gain of function mutations in the CXCR4 receptor gene that occur in patients with WHIM syndrome lead to increased responsiveness to CXCL12 and retention of leukocytes in the bone marrow. Mavorixafor inhibits the response to CXCL12 in both wild‑type and for mutated CXCR4 variants associated with WHIM syndrome. Treatment with mavorixafor results in increased mobilization of leukocytes and lymphocytes from the bone marrow into peripheral circulation.

Recommended dosage: Weight more than 50 kg: 400 mg orally once daily. Weight less than or equal to 50 kg: 300 mg orally once daily. Administer XOLREMDI on an empty stomach after an overnight fast, and at least 30 minutes before food.

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

Embryo-fetal toxicity: Expected to cause fetal harm. Advise women of reproductive potential to use effective contraception. QTc Interval Prolongation:: Correct any modifiable risk factors, assess QTc at baseline and monitor QTc during treatment as clinically indicated. XOLREMDI dose reduction or discontinuation may be required due to drug-drug interactions.

The following clinically significant adverse reactions are described elsewhere in the labeling: QTc Interval Prolongation The most common adverse reactions (›10% and at a frequency higher than placebo) were: thrombocytopenia, pityriasis, rash, rhinitis, epistaxis, vomiting, and dizziness.

Strong CYP3A4 inhibitors: Reduce XOLREMDI daily dosage. P-gp inhibitors or moderate CYP3A4 inhibitors: Monitor more frequently for XOLREMDI adverse reactions and reduce XOLREMDI daily dosage if necessary. Strong CYP3A4 Inducers: Avoid concomitant use. CYP3A4 or P-gp substrates: Monitor more frequently for substrate adverse reactions unless otherwise recommended.

Store XOLREMDI refrigerated at 2°C to 8°C (36°F to 46°F). Store in and dispense from original container to protect from moisture.