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Xolremdi

mavorixafor
Used for WHIM syndrome
Used for WHIM syndrome

Xolremdi (mavorixafor) is a CXCR4 antagonist. It's the first medication FDA-approved to treat WHIM syndrome (warts, hypogammaglobulinemia, infections, and myelokathexis), a rare genetic condition that affects the immune system. It can be taken by people ages 12 years and older. Typically, you take Xolremdi (mavorixafor) by mouth once daily in the morning on an empty stomach, after fasting through the night. Some of the most common side effects include rash, vomiting, and nosebleeds.

Reviewed by:
reviewer
Celine Kwan, PharmD Candidate
reviewer
Connie Zheng, PharmD
reviewer
Daisy Chau, PharmD
Last reviewed on December 19, 2024
BasicsSide effectsPros & consPharmacist tipsFAQsWarningsDosageCostContraindicationsReferencesPricesMedicare
basics-icon

What is Xolremdi (mavorixafor)?

What is Xolremdi (mavorixafor) used for?

  • WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome in people ages 12 years and older

How Xolremdi (mavorixafor) works

Xolremdi (mavorixafor) is a CXCR4 antagonist, meaning it blocks the activity of CXCR4 proteins. In a healthy immune system, these proteins tell white blood cells (WBCs) to stay in the bone marrow where they can grow and mature. When there’s an infection, CXCR4 proteins tell the WBCs to move into the bloodstream where they can fight off the infection.

People who have WHIM syndrome have a change in the CXCR4 gene that makes the CXCR4 proteins overly active. As a result, the WBCs stay trapped in the bone marrow and aren’t able to move into the bloodstream. Xolremdi (mavorixafor) treats WHIM by blocking the CXCR4 proteins so that the WBCs can leave the bone marrow to fight infections in the body.

Drug Facts

Common BrandsXolremdi
Drug ClassCXCR4-antagonist
Controlled Substance ClassificationNot a controlled medication
Generic StatusNo lower-cost generic available
AvailabilityPrescription only
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What are the side effects of Xolremdi (mavorixafor)?

The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.

Common Side Effects

Other Side Effects

  • Headache
  • Nausea
  • Dry skin
  • Dry eyes
  • Changes in taste after dose
  • Skin itching

Serious Side Effects

Contact your healthcare provider immediately if you experience any of the following.
  • Serious heart rhythm problem (long QT syndrome): pounding heart, fainting, tiredness, dizziness, seizures

Source: DailyMed

The following side effects have also been reported

Side effects that you should report to your care team as soon as possible:

  • Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
  • Heart rhythm changes—fast or irregular heartbeat, dizziness, feeling faint or lightheaded, chest pain, trouble breathing
  • Unusual bruising or bleeding

Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):

pros-and-cons

Pros and cons of Xolremdi (mavorixafor)

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Pros

First oral medication to treat WHIM syndrome

Only taken once a day

Can be taken by children as young as 12 years old

Can lower your risk of infections

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Cons

Must fast the night before, and can't eat food 30 minutes after taking a dose

Can’t take while pregnant or breastfeeding

Each dose consists of 3 to 4 capsules (each capsule contains 100 mg of mavorixafor)

pharmacist-tips

Pharmacist tips for Xolremdi (mavorixafor)

pharmacist

  • You’re recommended to take Xolremdi (mavorixafor) on an empty stomach after fasting overnight, and at least 30 minutes before eating. This helps your body fully absorb the medication. Eating before taking your medication might make it not work as well.

    • When you take Xolremdi (mavorixafor), swallow the capsules whole. Don’t open, break, or chew the capsules because doing this might affect how the medication works.

      • If you miss a dose of Xolremdi (mavorixafor), skip it and take your next dose as scheduled. Don’t take two doses on the same day. Try setting an alarm on your phone, using a pill box, or marking a checklist to remember to take your medication.

        • Store Xolremdi (mavorixafor) in the refrigerator, avoiding moisture. Keep the medication in the original container.

          • Avoid eating grapefruit or drinking grapefruit juice when you’re taking Xolremdi (mavorixafor). Grapefruits can interact with the medication and raise the risk of side effects.

            • Xolremdi (mavorixafor) can interact with several medications, which can increase your risk for side effects or make some medications work less well. Let your prescriber know about all the medications, supplements, and herbs you are taking before starting this medication

              • Some people taking Xolremdi (mavorixafor) have reported nosebleeds. Some ways to manage this include using a humidifier, applying petroleum jelly, and using saline sprays to decrease congestion. If the nosebleeds don’t go away, speak to your prescriber.

                • Xolremdi (mavorixafor) might cause allergy symptoms, like a stuffy nose, sore throat, or headache. If your symptoms are bothersome, ask your healthcare team whether over-the-counter antihistamines are appropriate for you.

                  faqs

                  Frequently asked questions about Xolremdi (mavorixafor)

                  What is WHIM syndrome?
                  WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is a rare genetic condition. People with WHIM have an immune system that doesn't work properly. They have white blood cells (WBCs) that get stuck in the bone marrow (myelokathexis) and lower levels of antibodies in the blood (hypogammaglobulinemia). As a result, they are more prone to infections, including warts caused by viruses. Xolremdi (mavorixafor) treats WHIM by helping WBCs move from the bone marrow into the bloodstream where they can fight infections.
                  Does Xolremdi (mavorixafor) cure WHIM?
                  No, Xolremdi (mavorixafor) doesn’t cure WHIM because it doesn’t fix the CXCR4 gene. But rather, it blocks the overactive CXCR4 protein so that the immune cells can move into the bloodstream to defend against germs like bacteria or viruses. As a result, people who take Xolremdi (mavorixafor) might be better able to fight infections. Xolremdi (mavorixafor) is the first medication that's FDA-approved specifically to treat WHIM by targeting CXCR4. Prior treatment options involved managing or preventing specific symptoms of WHIM syndrome, such as raising the number of antibodies with immunoglobulin replacement therapy.
                  How well does Xolremdi (mavorixafor) work to treat WHIM?
                  A year-long study looked at how well Xolremdi (mavorixafor) works by measuring how long white blood cell (WBC) counts stayed above levels that reduce the risk of infections. Results showed that people who took Xolremdi (mavorixafor) had WBC counts above this level for much longer compared to those who took placebo. People who took Xolremdi (mavorixafor) also had fewer infections and infections that were less severe compared to those who took placebo. Data suggest that Xolremdi (mavorixafor) isn’t likely to improve warts, but more research is needed in this area. Talk with your healthcare team if you have more questions about how well this medication works.
                  Does Xolremdi (mavorixafor) interact with supplements?
                  Yes, Xolremdi (mavorixafor) can interact with certain supplements. For example, taking St. John’s Wort can lower the level of Xolremdi (mavorixafor) in your body and cause it to not work as well. Additionally, taking goldenseal can raise the level of Xolremdi (mavorixafor) and put you at higher risk for side effects like nosebleeds. Tell your healthcare team what vitamins and supplements you are taking so they can help you check for interactions.
                  Can I take Xolremdi (mavorixafor) if I am pregnant?
                  No, you shouldn’t take Xolremdi (mavorixafor) if you’re pregnant. Taking this medication during pregnancy might cause harm to your unborn baby. Before starting, your healthcare team will ask you to take a pregnancy test to double check your pregnancy status. If you're able to become pregnant, they’ll recommend effective birth control when taking Xolremdi (mavorixafor) and for 3 weeks after your last dose.
                  Can I take Xolremdi (mavorixafor) if I am breastfeeding?
                  You should avoid breastfeeding while taking Xolremdi (mavorixafor). There’s not enough research to know how this medication might affect breastfed babies. But because there’s a risk of potentially serious side effects, you shouldn’t breastfeed during treatment and for 3 weeks after finishing your last dose.
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                  What are the risks and warnings for Xolremdi (mavorixafor)?

                  Xolremdi (mavorixafor) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.

                  risk-warning

                  Harm to unborn baby

                  Due to the way Xolremdi (mavorixafor) works, it might cause harm to unborn babies if taken by mothers during pregnancy. To be safe, people who are pregnant shouldn’t take this medication. If you’re able to become pregnant, your healthcare team will ask you to take a pregnancy test before starting treatment to see if you’re pregnant. Additionally, it’s important that you use an effective form of birth control while taking Xolremdi (mavorixafor) and for 3 weeks after your last dose.

                  risk-warning

                  Irregular heart rhythms

                  • Risk factors: Older age | Female | Low blood potassium levels | Taking other medications that affect heart rhythm

                    Xolremdi (mavorixafor) can cause a serious heart rhythm problem called long QT syndrome. Before starting this medication, your healthcare team will order an electrocardiogram (ECG) to monitor your heart rate and rhythm. Monitoring is important because people with this condition often don’t notice any symptoms. If you're taking other medications that can cause this side effect, your care team might order ECGs more often during treatment. If needed, your care team will lower your dose of Xolremdi (mavorixafor) to prevent heart rhythm problems. Get medical help right away if you notice symptoms of heart rhythm problems, such as pounding heart, tiredness, dizziness, and fainting.

                    dosage

                    Xolremdi (mavorixafor) dosage forms

                    Typical dosing for Xolremdi (mavorixafor)

                    Your healthcare team will determine your dose of Xolremdi (mavorixafor) based on your weight.

                    In general:

                    • People who weigh more than 50 kg: The typical dose is 400 mg by mouth in the morning once daily on an empty stomach, after fasting through the night.

                    • People who weigh 50 kg or less: The typical dose 300 mg by mouth in the morning once daily on an empty stomach, after fasting through the night.

                    Your dose might be different if you're taking another medication that can interact with Xolremdi (mavorixafor).

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                    contraindications-icon

                    Xolremdi (mavorixafor) contraindications

                    Contraindications are specific health conditions or situations in which a person should not take a medication due to safety concerns. If you have any of the following conditions or if any of the following apply to you, let your healthcare provider know because Xolremdi (mavorixafor) will not be safe for you to take.

                    • Also taking medications that mainly depend on the liver enzyme CYP2D6 to clear out of the body

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                    References

                    Best studies we found

                    Badolato, R., et al. (2024). A phase 3 randomized trial of mavorixafor, a CXCR4 antagonist, for WHIM syndrome. Blood.

                    de Groot, M. J., et al. (2009). Understanding CYP2D6 interactions. Drug Discovery Today.

                    Geier, C. B. (2024). Mavorixafor: A new hope for WHIM syndrome. Blood.

                    View All References (11)

                    Hoy, S. M. (2024). Mavorixafor: First approval. Drugs.

                    Huq, M. E., et al. (2023). Hypogammaglobulinemia. StatPearls.

                    Immune Deficiency Foundation. (n.d.). WHIM syndrome.

                    MedlinePlus. (2015). CXCR4 gene.

                    Nadeem, F., et al. (2024). Xolremdi (Mavorixafor): A breakthrough in WHIM syndrome treatment - unraveling efficacy and safety in a rare disease frontier. Annals of Medicine and Surgery.

                    Nahid, N. A., et al. (2022). CYP2D6 pharmacogenetics and phenoconversion in personalized medicine. Expert Opinion on Drug Metabolism & Toxicology.

                    National Center for Complementary and Integrative Health. (2021). Goldenseal.

                    National Institute of Allergy and Infectious Diseases. (2019). Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome.

                    National Organization for Rare Disorders. (2024). WHIM syndrome.

                    U.S. Food and Drug Administration. (2024). FDA approves first drug for WHIM syndrome, a rare disorder that can lead to recurrent, life-threatening infections.

                    X4 Pharmaceuticals, Inc. (2024). Xolremdi- mavorixafor capsule, gelatin coated [package insert]. DailyMed.

                    GoodRx Health has strict sourcing policies and relies on primary sources such as medical organizations, governmental agencies, academic institutions, and peer-reviewed scientific journals. Learn more about how we ensure our content is accurate, thorough, and unbiased by reading our editorial guidelines.
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