Xolremdi

Pronunciation: Zoll-REM-dee
Generic name: mavorixafor
Dosage form: oral capsule

Medically reviewed by Carmen Pope, BPharm. Last updated on Apr 30, 2024.

What is Xolremdi?

Xolremdi (mavorixafor) is an oral CXC chemokine receptor 4 antagonist that may be used to increase the number of circulating mature neutrophils and lymphocytes in adults and children 12 years of age and older with WHIM syndrome (warts, hypogammaglobulinemia, infections, and myelokathexis).

WHIM syndrome is a rare genetic disorder affecting the immune system. It's caused by a malfunction in the CXCR4/CXCL12 pathway, leading to white blood cells being stuck in the bone marrow instead of circulating properly. This results in low levels of certain immune cells, making affected individuals prone to frequent infections. They also commonly experience stubborn warts caused by HPV, have reduced antibody production, and face an elevated risk of certain cancers.

Xolremdi (mavorixafor) works by inhibiting the binding of a specific protein called SDF-1α/CXCL12 to the CXCR4 receptor, which is involved in immune cell movement. By inhibiting this binding Xolremdi helps prevent immune cells from being trapped in the bone marrow. This is particularly relevant in conditions like WHIM syndrome, where mutations in the CXCR4 gene cause immune cells to be overly responsive to CXCL12, leading to their retention in the bone marrow. Xolremdi works in both normal and mutated CXCR4 variants associated with WHIM syndrome, promoting the release of immune cells from the bone marrow into the bloodstream.

Xolremdi was FDA-approved on 26 April 2024.

Xolremdi side effects

Xolremdi can cause serious side effects, see warnings below.

The most common side effects affecting 10% or more people taking Xolremdi were:

Low platelet counts (thrombocytopenia)
A rash, such as pityriasis
Nasal congestion, sneezing, and stuffiness
Nosebleeds
Vomiting
Dizziness.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to X4 Pharmaceuticals, Inc. at 1-866-MED-X4MI (1-866-633-9464) or the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Warnings

Should not be given medications that are highly dependent on CYP2D6 enzymes for clearance, such as fluoxetine or amitriptyline.

May cause QTc interval prolongation.

May cause harm to an unborn baby. Women who can bear children should use effective contraception while taking Xolremdi and for 3 weeks after the last dose.

Can prolong the QTc interval. Your healthcare provider should correct any modifiable risk factors (such as low potassium levels), assess your QTc before starting treatment, and monitor it throughout treatment. The risk of QTc prolongation is higher in those receiving other QTc prolonging drugs, such as haloperidol, amiodarone, sotalol, or ondansetron, or taking other medications that increase blood levels of Xolremdi.

Should not be used by people with severe kidney disease, end-stage kidney disease, or moderate to severe liver disease.

Before taking

Before taking Xolremdi, tell your doctor if you:

Have allergies
Have preexisting heart disease, such as an arrhythmia or QTc prolongation
Take other medications
Have low platelet levels
Have skin conditions
Have frequent nose bleeds
Have liver or kidney disease
Are pregnant, may become pregnant, or intend to become pregnant
Are breastfeeding.

Pregnancy

You may need to have a negative pregnancy test before starting this treatment. Do not use Xolremdi if you are pregnant.

Xolremdi may cause fetal harm and abnormal placental development. Females with childbearing potential should use effective contraception while receiving Xolremdi and for 3 weeks after the last dose. If you inadvertently become pregnant while receiving Xolremdi, tell your doctor right away.

Breastfeeding

You should not breastfeed while taking Xolremdi and for 3 weeks after the last dose. Talk to your healthcare provider about the best way to feed your baby.

How is Xolremdi administered?

Xolremdi is usually administered once a day before breakfast.

Administer on an empty stomach after an overnight fast, and at least 30 minutes before food.

The usual dosage of Xolremdi is based on weight:

more than 50 kg: 400 mg orally once daily.
less than or equal to 50 kg: 300 mg orally once daily.

What should I avoid while taking Xolremdi?

Advise eating or drinking products with grapefruit, as grapefruit is a strong CYP3A4 inhibitor and may increase the risk of side effects from Xolremdi.

What other drugs will affect Xolremdi?

Other drugs may interact with Xolremdi, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all your current medicines and any medicine you start or stop using. Especially tell your doctor if you take:

Goldenseal or grapefruit as it may increase the risk of side effects from Xolremdi.
St. John’s Wort, as it may reduce the efficacy of Xolremdi.
Medications that are highly dependent on CYP2D6 enzymes for clearance, such as fluoxetine or amitriptyline
Medications that cause low potassium, such as diuretics
QTc prolonging drugs, such as haloperidol, amiodarone, sotalol, or ondansetron
Strong CYP3A4 inhibitors, such as itraconazole
CYP3A4 substrates, such as midazolam
P-gp substrates, such as digoxin or metformin.

This is not a complete list of interactions. Talk to your healthcare provider or pharmacist about drug interactions. See the Xolremdi Package Insert for more information.

Storage

Store Xolremdi in the refrigerator at 2°C to 8°C (36°F to 46°F). Keep it in the original container to protect it from moisture and tightly closed.

Ingredients

Active: mavorixafor 100mg.

Inactive: colloidal silicon dioxide, croscarmellose sodium, dibasic calcium phosphate dihydrate, microcrystalline cellulose, sodium lauryl sulfate, and sodium stearyl fumarate.

Hard gelatin capsule: FD&C Blue #2, gelatin, and titanium dioxide.

Black Ink: ammonium hydroxide 28%, ferrosoferric oxide/black iron oxide (E172), isopropyl alcohol, n-butyl alcohol, propylene glycol, and shellac glaze in ethanol.