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RecruitingNCT06056297Updated Aug 6, 2026

A Study of Mavorixafor in Participants With Congenital and Acquired Primary Autoimmune and Idiopathic Chronic Neutropenic Disorders Who Are Experiencing Recurrent and/or Serious Infections

A Phase 3 interventional study of Mavorixafor and Placebo in Neutropenia, sponsored by X4 Pharmaceuticals. Recruiting at 114 sites in 25 countries. Open to participants aged 12 Years and older. Per ClinicalTrials.gov, last updated 2026-08-06.

Sponsored by X4 Pharmaceuticals · Phase 3, Interventional, and Treatment

Phase
Phase 3
Study type
Interventional
Enrollment
176
Allocation
Randomized
Ages
12 Years and older
Sex
All
01

Study summary

The purpose of this study is to demonstrate the efficacy and evaluate the safety and tolerability of mavorixafor in participants with congenital or acquired primary autoimmune and idiopathic chronic neutropenic disorders who are experiencing recurrent and/or serious infections as assessed by demonstrating its clinical benefit and increasing levels of circulating neutrophils.

Read the detailed description

All participants will continue their pre-study background therapy, defined as the participant's current treatment regimen. Options include, but are not limited to, granulocyte-colony stimulating factor (G-CSF), immunoglobulin replacement therapy, prophylactic antibiotics, or "watchful waiting".

02

Conditions studied

  • Neutropenia

Keywords

  • Mavorixafor
  • Chronic neutropenia
  • Chronic idiopathic neutropenia
  • Severe Congenital Neutropenia (SCN)
  • C-X-C chemokine receptor 4 (CXCR4)
  • Congenital and acquired neutropenia
  • Autoimmune disease
  • Cohen syndrome
  • Barth syndrome
  • ELANE
  • G6PC3
  • GSD1b
  • Kostmann disease
  • Shwachman-Diamond syndrome
  • Autoimmune neutropenia (AIN)
03

Who can participate

Ages eligible
12 Years and older
Sexes eligible
All
Accepts healthy volunteers
No

Eligibility criteria

Key Inclusion Criteria:

  • Diagnosis of congenital or acquired primary autoimmune and idiopathic chronic neutropenic disorder ≥6 months prior to the screening visit that is not attributable to medications, active or recent infections or malignancy.
  • Congenital Neutropenia, including but not limited to these classifications:

    1. Isolated with a permanent (non-cyclic) presentation, for example, elastase, neutrophil expressed (ELANE), colony stimulating factor 3 receptor (CSF3R), C-X-C chemokine receptor 2 (CXCR2), Wiskott-Aldrich syndrome (WAS)
    2. Associated with extra-hematologic manifestations, for example, Barth syndrome, Cohen syndrome, glucose-6-phosphatase catalytic subunit 3 (G6PC3), Kostmann disease
    3. Associated with metabolic disorders, for example, glycogen storage disease 1b (GSD1b)
    4. Shwachman-Diamond syndrome
  • Acquired Primary Neutropenia

    1. Chronic idiopathic neutropenia
    2. Primary autoimmune neutropenia. Other chronic neutropenia (CN) disorders that may be eligible for enrollment can be clarified and approved upon discussion with study Medical Monitor.
  • Have an ANC \<1000 cells/µL during screening (single ANC value from hematology) and confirmed trough mean ANC (mean value of multiple ANC measurements over 6 hours) at baseline visit, with no clinical evidence of systemic infection.
  • Prior history of recurrent and/or serious infections during the 12 months preceding the screening visit (that is, suffering sequelae of chronic neutropenia), as defined by having at least 2 infections in the last 12 months that meet the following criteria:
  • Infection requiring the use of antibiotics (intravenous [IV]/oral); OR
  • Infection requiring a visit to healthcare facility (including but not limited to emergency room visit, urgent care facility, primary care physician's office, or in-patient hospitalization);

AND for all potential participants:

  • Infections considered by the Investigator to be likely related to the potential participant's CN disorder.
  • Participants who are on G-CSF or other active background therapy must have been receiving these therapies during the previous 12 months while continuing to suffer from infections, be on a stable dose and dosing schedule for ≥4 weeks prior to screening visit and remain on this dose and dosing schedule throughout the study (unless ANC >10,000 cells/µL for ≥4 weeks).
  • Participants must be willing to keep their G-CSF or other background therapy doses/regimens stable (other than for safety reasons) for the duration of the study.

Key Exclusion Criteria:

  • A diagnosis of secondary neutropenia including those due to:

    1. Hypersplenism
    2. Infection
    3. Malignancy
    4. Autoimmune disease, for example, systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel disease, graft-versus-host disease, thyroid disease
    5. Nutritional deficiency, for example, vitamin B12, folic acid, copper, caloric malnutrition
    6. Drug-induced cause, for example, chemotherapy, clozapine, antiretrovirals, antibiotics, monoclonal antibodies.
  • A diagnosis of any of the following:

    1. Aplastic anemia
    2. Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome
    3. Certain CNs, including but not limited to these classifications are excluded:

      1. Isolated with a cyclic presentation, for example, elastase, neutrophil expressed (ELANE)
      2. Associated with immune dysregulation, for example, common variable immunodeficiency (CVID), autoimmune lymphoproliferative syndrome (ALPS), familial hemophagocytic lymphohistiocytosis, Chédiak-Higashi syndrome, GATA-binding protein 2 (GATA2) deficiency syndrome
      3. Associated with bone marrow failure, for example, Fanconi anemia, Diamond-Blackfan anemia
    4. Neutropenia associated with a Duffy-null phenotype (formerly known as benign ethnic neutropenia). However, a participant with an autosomal dominant pathogenic variant in a gene associated with CN on a Duffy-null background may be eligible for inclusion
  • A medical or personal condition that may potentially compromise the safety of the participant, may preclude the participant's successful completion of the clinical study, or could, in the opinion of the Investigator or the Medical Monitor, interfere with the objectives of the study.
  • Received more than 1 dose of mavorixafor in the past.
  • Received C-X-C chemokine receptor 4 (CXCR4) antagonist (other than mavorixafor) in the past 6 months.
  • Participants taking pegylated-G-CSF unless they have a diagnosis of congenital neutropenia confirmed at screening.
  • Participant is currently taking or has taken other investigational drug \<30 days prior to the screening visit or 5 half-lives, whichever is longer.

Note: Other protocol-defined inclusion and exclusion criteria may apply.

04

Study design

Phase
Phase 3
Primary purpose
Treatment
Allocation
Randomized
Intervention model
Parallel assignment
Masking
Quadruple (Participant, Care provider, Investigator, Outcomes assessor)
Enrollment
176 participants (estimated)

Study arms

  • Experimental
    Mavorixafor

    Participants will receive mavorixafor orally once daily starting from Day 1 through Week 52.

    Drug: Mavorixafor

  • Placebo comparator
    Placebo

    Participants will receive placebo to match mavorixafor orally once daily starting from Day 1 through Week 52.

    Drug: Placebo

Interventions

  • DrugMavorixafor

    Mavorixafor will be administered per schedule specified in the arm description.

    Also known as: X4P-001

  • DrugPlacebo

    Placebo will be administered per schedule specified in the arm description.

05

What researchers measure

Primary outcomes

  1. Co-primary Endpoint: Annualized Infection Rate Based on Infections Adjudicated by Blinded Infection Adjudication Committee (BIAC) During the Treatment Period

    Time frame: Up to 52 Weeks

  2. Co-primary Endpoint: Number of Participants Meeting the Definition of a Positive Absolute Neutrophil Count (ANC) Response

    Positive ANC response: Increase of ANC \>500 cells/microliter (µL) from baseline.

    Time frame: Up to 52 weeks

Secondary outcomes

  1. Infection Severity Based on Common Terminology Criteria for Adverse Events (CTCAE) Adjudicated by a BIAC During the Treatment Period

    Time frame: Up to 52 Weeks

  2. Infection Duration Based on Duration of Infections Adjudicated by a BIAC During the Treatment Period in Those Participants who Developed Infections

    Time frame: Up to 52 Weeks

  3. Antibiotic Use Due to Infection, Characterized by the Frequency of Antibiotic Use During the Treatment Period

    Time frame: Up to 52 Weeks

  4. Oral Ulcers, as Assessed by Presence or Absence of Ulcers During the Treatment Period

    Time frame: Up to 52 Weeks

  5. Change From Baseline in Patient Reported Outcomes Measurement Information System Short Form (PROMIS SF) Fatigue Questionnaire Total Score

    Time frame: Baseline, Week 52

06

Study locations

107 of 114 sites recruiting
  • Phoenix Children's Hospital
    Phoenix, Arizona 85016, United States
    Recruiting
  • UC Irvine
    Irvine, California 92697, United States
    Recruiting
  • Orso Health
    La Jolla, California 92037, United States
    Recruiting
  • University of California, Los Angeles-UCLA
    Los Angeles, California 90095, United States
    Recruiting
  • Orso Health
    Torrance, California 90505, United States
    Withdrawn
  • University of Southern Florida
    St. Petersburg, Florida 33701, United States
    Recruiting
  • Children's Healthcare of Atlanta (CHOA)
    Atlanta, Georgia 30322, United States
    Recruiting
  • University of Iowa
    Iowa City, Iowa 52242, United States
    Recruiting
  • Boston Children's Cancer Center
    Boston, Massachusetts 02143, United States
    Recruiting
  • University of Michigan
    Ann Arbor, Michigan 48109, United States
    Recruiting
  • Washington University
    St Louis, Missouri 63130, United States
    Recruiting
  • Duke University Medical Center
    Durham, North Carolina 27710, United States
    Recruiting
  • Cincinnati Children's Hospital Medical Center
    Cincinnati, Ohio 45229, United States
    Recruiting
  • Cleveland Clinic
    Cleveland, Ohio 44195, United States
    Recruiting
  • The Children's Hospital of Philadelphia
    Philadelphia, Pennsylvania 19104, United States
    Recruiting
  • St. Jude Childrens; Research Hospital
    Memphis, Tennessee 38105, United States
    Recruiting
  • UT Southwestern Medical Center
    Dallas, Texas 75390, United States
    Recruiting
  • Cook Children's Medical Center
    Fort Worth, Texas 76104, United States
    Recruiting
  • Texas Children's Hospital/ Baylor College of Medicine
    Houston, Texas 77030, United States
    Recruiting
  • Sarah Cannon Research Institute
    Woodland, Texas 77380, United States
    Recruiting
  • University of Utah
    Salt Lake City, Utah 84112, United States
    Recruiting
  • Froedtert And Medical College Of Wisconsin
    Milwaukee, Wisconsin 53226, United States
    Recruiting
  • Hospital El Cruce
    Buenos Aires, B1888AAE, Argentina
    Recruiting
  • Instituto de Investigaciones Clínicas Córdoba
    Córdoba, X5000AAW, Argentina
    Recruiting
  • Santorio 9 de Julio
    San Miguel de Tucumán, T4000IIH, Argentina
    Recruiting
  • Perth Children's Hospital
    Nedlands, 6009, Australia
    Recruiting
  • Icon Cancer Centre Southport
    Southport, 4215, Australia
    Recruiting
  • Ballarat Oncology And Haematology Services
    Wendouree, 3355, Australia
    Recruiting
  • Universitair Ziekenhuis Leuven
    Leuven, 3000, Belgium
    Recruiting
  • University of Alberta Hospital
    Edmonton, AB T6G 2R3, Canada
    Recruiting
  • BC Children's Hospital
    Vancouver, V5Z 4H4, Canada
    Recruiting
  • Fundacion Cardiovascular de Colombia-Instituto del Corazon Florida Blanca
    Floridablanca, Santander Department 681003, Colombia
    Recruiting
  • Clinica IMBANACO S.A.S
    Cali, 76004, Colombia
    Recruiting
  • Clínica del Country
    Chapinero, 0813, Colombia
    Recruiting
  • Hospital Pablo Tobón Uribe
    Medellín, 500034, Colombia
    Recruiting
  • The University Hospital Brno
    Brno, 62500, Czechia
    Recruiting
  • Motol University Hospital
    Prague, 15006, Czechia
    Recruiting
  • Institute of Hematology and Pediatric Oncology - Lyon
    Lyon, 69008, France
    Recruiting
  • Hôpital Saint Louis
    Paris, 75010, France
    Recruiting
  • Hospital Armand Trousseau Ap-Hp
    Paris, 75012, France
    Recruiting
  • Hôpital Necker - Enfants Malade
    Paris, 75015, France
    Recruiting
  • Centre Hospitalier Universitaire de Rennes
    Rennes, 75033, France
    Recruiting
  • Institut Universitaire de Cancérologie de Toulouse - Oncopôle
    Toulouse, 31059, France
    Recruiting
  • LTD "Israeli-Georgian Medical Research Clinic Healthycore"
    Tbilisi, 112, Georgia
    Recruiting
  • LEPL The First University Clinic of Tbilisi State Medical University
    Tbilisi, 141, Georgia
    Recruiting
  • JSC K. Eristavi National Center of Experimental and Clinical Surgery
    Tbilisi, 159, Georgia
    Recruiting
  • LTD Multiprofile Clinic Concilium Medulla
    Tbilisi, 186, Georgia
    Recruiting
  • Klinik und Poliklinik fur Kinder- und Jugendmedizin, Universitatsklinikum Carl Gustav Carus an der TU
    Dresden, 01307, Germany
    Recruiting
  • Universitaetsklinikum Carl Gustav Carus Tu Dresden
    Dresden, 01307, Germany
    Recruiting
  • Hellenic Airforce 251 General Hospital
    Athens, 10177, Greece
    Recruiting
  • University General Hospital of Heraklion
    Heraklion, 71500, Greece
    Recruiting
  • The Olympion Private General Clinic
    Pátrai, 26443, Greece
    Recruiting
  • AHEPA General Hospital of Thessaloniki
    Thessaloniki, 54636, Greece
    Recruiting
  • CMC Deli Klinika
    Budapest, 1012, Hungary
    Recruiting
  • Semmelweis University
    Budapest, 1085, Hungary
    Recruiting
  • Teaching Hospital Markusovszky
    Szombathely, 9700, Hungary
    Recruiting
  • Indira Gandhi Institute Of Medical Sciences
    Patna, Bihar 800014, India
    Withdrawn
  • Nirmal Hospital Private Limited
    Surat, Gujarat 395002, India
    Withdrawn
  • Pacific Medical College and Hospital
    Udaipur, Rajasthan 313001, India
    Withdrawn
  • Yashoda Hospital
    Hyderabad, Telangana 500084, India
    Terminated
  • Ruth Rappaport Children's Hospital, Rambam Health Corporation
    Haifa, 3109601, Israel
    Recruiting
  • Hadassah University Hospital-ein Kerem
    Jerusalem, 9112001, Israel
    Recruiting
  • Schneider Children's Medical Center of Israel
    Petah Tikva, 4920235, Israel
    Recruiting
  • The Chaim Sheba Medical Center
    Ramat Gan, 5265601, Israel
    Recruiting
  • A. O. U. delle Marche
    Torrette, Ancona 60126, Italy
    Recruiting
  • ASST Spedali Civili di Brescia
    Brescia, 25121, Italy
    Recruiting
  • IRCCS Instituto G. Gaslini
    Genova, 16147, Italy
    Recruiting
  • Istituto Scientifico Romagnolo Per Lo Studio E La Cura Dei Tumori (IRST) Dino Amadori
    Meldola, 47014, Italy
    Recruiting
  • IRCCS San Gerardo Dei Tintori
    Monza, 20900, Italy
    Recruiting
  • Bambino Gesu Children's Hospital
    Roma, PC 00165, Italy
    Recruiting
  • AOU Città della salute e della scienza di Torino
    Torino, 10126, Italy
    Recruiting
  • Hospital Pakar Kanak-Kanak UKM
    Kuala Lumpur, 56000, Malaysia
    Recruiting
  • Unidade Local de Saúde da Região de Aveiro
    Aveiro, 3810-164, Portugal
    Recruiting
  • Unidade Local de Saude Coimbra - Paediatric Hospital
    Coimbra, 3000-602, Portugal
    Recruiting
  • Unidade Local de Saude Coimbra - Quinta dos Vales
    Coimbra, 3041-801, Portugal
    Recruiting
  • Unidade Local de Saude Santa Maria
    Lisbon, 1649-028, Portugal
    Recruiting
  • Spitalul Clinic Colentina Site 1
    Bucharest, 020125, Romania
    Recruiting
  • Spitalul Clinic Colentina Site 2
    Bucharest, 020125, Romania
    Recruiting
  • Institutul Clinic Fundeni
    Bucharest, 022328, Romania
    Recruiting
  • Institutul Clinic Fundeni
    Bucharest, 22328, Romania
    Recruiting
  • Spitalul Clinic Județean de Urgență
    Târgu Mureş, 540136, Romania
    Recruiting
  • University Clinical Centre of Serbia
    Belgrade, 11000, Serbia
    Recruiting
  • ICO Badalona - Institut Català d'Oncologia
    Barcelona, PC 08916, Spain
    Recruiting
  • Hospital Del Mar
    Barcelona, PC08003, Spain
    Recruiting
  • Hospital San Pedro De Alcantara
    Cáceres, 10003, Spain
    Recruiting
  • Hospital Universitario Reina Sofía
    Córdoba, 14004, Spain
    Withdrawn
  • Hospital Universitario de Gran Canaria Doctor Negrín
    Las Palmas de Gran Canaria, 35010, Spain
    Recruiting
  • Hospital De La Princesa
    Madrid, 28006, Spain
    Recruiting
  • Hospital Universitario Infanta Leonor
    Madrid, 28031, Spain
    Withdrawn
  • Hospital Universitario Fundacion Jimenez Diaz
    Madrid, 28040, Spain
    Recruiting
  • Hospital Universitario Marqués de Valdecilla
    Santander, 39008, Spain
    Recruiting
  • Hospital Universitario Virgen del Rocio
    Seville, 41013, Spain
    Recruiting
  • Hospital Universitario y Politecnico de la Fe
    Valencia, 46026, Spain
    Recruiting
  • Inselspital - University Hospital Bern Site 1
    Bern, 3010, Switzerland
    Recruiting
  • Inselspital - University Hospital Bern Site 2
    Bern, 3010, Switzerland
    Recruiting
  • Ramathibodi Hospital
    Bangkok, 10400, Thailand
    Recruiting
  • Siriraj Hospital
    Bangkok, 10700, Thailand
    Recruiting
  • Namik Kemal University (Tekirdağ Namık Kemal Üniversitesi)
    Tekirdağ, Süleymanpaşa 59030, Turkey (Türkiye)
    Recruiting
  • Dr. Abdurrahman Yurtaslan Ankara Oncology Training And Research Hospital
    Ankara, 6000, Turkey (Türkiye)
    Recruiting
  • Ankara University School of Medicine
    Ankara, 6620, Turkey (Türkiye)
    Recruiting

Showing the first 100 of 114 sites across 25 countries.

07

References and documents

Individual participant data

Plan to share: No

No publications or documents are linked to this record.

08

Registry details

Key details

Study ID
NCT06056297
Lead sponsor
X4 Pharmaceuticals
Responsible party
Sponsor
First posted
Sep 28, 2023
Start date
Jun 6, 2024
Primary completion
Sep 2027 (estimated)
Completion
Nov 2027 (estimated)
Last update
Aug 6, 2026

Study contacts

Patient Affairs and Advocacy
Contact
clinicaltrialinfo@x4pharma.com
857-529-5779
Vice President Global Head of Clinical Development and Safety
study director · X4 Pharmaceuticals

Oversight

Data monitoring committee
Yes
FDA-regulated drug
Yes
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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