WHIM syndrome

Active Ingredient: Mavorixafor

Indication for Mavorixafor

Population group: only adolescents (12 years - 18 years old) , adults (18 - 65 years old)
Therapeutic intent: Curative procedure

Mavorixafor is indicated in patients 12 years of age and older for the treatment of WHIM syndrome (warts, hypogammaglobulinemia, infections and myelokathexis) to increase the number of circulating mature neutrophils and lymphocytes.

For this indication, competent medicine agencies globally authorize below treatments:

For patients weighting >50 kg 400 mg once daily and for patients weighting ≤50 kg 300 mg once daily

For:

Dosage regimens

Regimen A, in the case that patient weight is > 50 kg :

Oral, 400 milligrams mavorixafor, once daily -30 minutes before breakfast.

Regimen B, in the case that patient weight is ≤ 50 kg :

Oral, 300 milligrams mavorixafor, once daily -30 minutes before breakfast.

Detailed description

The recommended dose is:

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

Elderly

There are limited data on patients aged 65 years and older.

Missed dose

If a dose is missed, the next dose should be taken as scheduled. The patient should not take a double dose to make up for a missed dose.

Dose modifications

Concomitant use of mavorixafor with strong or moderate CYP3A4 inhibitors When used concomitantly with a strong CYP3A4 inhibitor, daily dose should be reduced to 200 mg.

Concomitant use of mavorixafor with strong or moderate CYP3A4 inhibitors

When used concomitantly with a strong CYP3A4 inhibitor, daily dose should be reduced to 200 mg. When used concomitantly with a moderate CYP3A4 inhibitor, adverse reactions that may be associated with an increase in mavorixafor exposure should be monitored more frequently, and the mavorixafor daily dose should be reduced by steps of 100 mg, as clinically necessary, but not to a dose less than 200 mg.

Concomitant use of mavorixafor with P-gp inhibitors

When used concomitantly with a P-gp inhibitor, adverse reactions that may be associated with an increase in mavorixafor exposure should be monitored more frequently, and the mavorixafor daily dose should be reduced by steps of 100 mg, as clinically necessary, but not to a dose less than 200 mg.

Active ingredient

Mavorixafor

Mavorixafor is a CXC Chemokine Receptor 4 (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. Treatment with mavorixafor results in increased mobilisation of neutrophils and lymphocytes and monocytes from the bone marrow into peripheral circulation.

Read more about Mavorixafor

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