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

NCT ID: NCT06056297

Last Updated: 2025-11-14

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Clinical Phase

PHASE3

Total Enrollment

176 participants

Study Classification

INTERVENTIONAL

Study Start Date

2024-06-06

Study Completion Date

2027-11-30

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

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.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

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".

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Neutropenia

Keywords

Explore important study keywords that can help with search, categorization, and topic discovery.

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)

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

Mavorixafor

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

Group Type EXPERIMENTAL

Mavorixafor

Intervention Type DRUG

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

Placebo

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

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

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

Interventions

Learn about the drugs, procedures, or behavioral strategies being tested and how they are applied within this trial.

Mavorixafor

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

Intervention Type DRUG

Placebo

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

Intervention Type DRUG

Other Intervention Names

Discover alternative or legacy names that may be used to describe the listed interventions across different sources.

X4P-001

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

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.

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.
Minimum Eligible Age

12 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

X4 Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Responsibility Role SPONSOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Vice President Global Head of Clinical Development and Safety

Role: STUDY_DIRECTOR

X4 Pharmaceuticals

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Phoenix Children's Hospital

Phoenix, Arizona, United States

Site Status RECRUITING

UC Irvine

Irvine, California, United States

Site Status RECRUITING

Orso Health

La Jolla, California, United States

Site Status RECRUITING

University of California, Los Angeles-UCLA

Los Angeles, California, United States

Site Status RECRUITING

Orso Health

Torrance, California, United States

Site Status RECRUITING

University of Southern Florida

St. Petersburg, Florida, United States

Site Status RECRUITING

University of Iowa

Iowa City, Iowa, United States

Site Status RECRUITING

University of Michigan

Ann Arbor, Michigan, United States

Site Status RECRUITING

Washington University

St Louis, Missouri, United States

Site Status RECRUITING

Duke University Medical Center

Durham, North Carolina, United States

Site Status RECRUITING

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status RECRUITING

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

UT Southwestern Medical Center

Dallas, Texas, United States

Site Status RECRUITING

Cook Children's Medical Center

Fort Worth, Texas, United States

Site Status RECRUITING

University of Utah

Salt Lake City, Utah, United States

Site Status RECRUITING

Froedtert And Medical College Of Wisconsin

Milwaukee, Wisconsin, United States

Site Status RECRUITING

Hospital El Cruce

Buenos Aires, , Argentina

Site Status RECRUITING

Instituto de Investigaciones Clínicas Córdoba

Córdoba, , Argentina

Site Status RECRUITING

Santorio 9 de Julio

San Miguel de Tucumán, , Argentina

Site Status RECRUITING

Perth Children's Hospital

Nedlands, , Australia

Site Status RECRUITING

Icon Cancer Centre Southport

Southport, , Australia

Site Status RECRUITING

Ballarat Oncology And Haematology Services

Wendouree, , Australia

Site Status RECRUITING

University of Alberta Hospital

Edmonton, , Canada

Site Status RECRUITING

Clinica IMBANACO S.A.S

Cali, , Colombia

Site Status RECRUITING

The University Hospital Brno

Brno, , Czechia

Site Status RECRUITING

Motol University Hospital

Prague, , Czechia

Site Status RECRUITING

Institute of Hematology and Pediatric Oncology - Lyon

Lyon, , France

Site Status RECRUITING

Hôpital Saint Louis

Paris, , France

Site Status RECRUITING

Hospital Armand Trousseau Ap-Hp

Paris, , France

Site Status RECRUITING

Hôpital Necker - Enfants Malade

Paris, , France

Site Status RECRUITING

Centre Hospitalier Universitaire de Rennes

Rennes, , France

Site Status RECRUITING

LTD "Israeli-Georgian Medical Research Clinic Healthycore"

Tbilisi, , Georgia

Site Status RECRUITING

LEPL The First University Clinic of Tbilisi State Medical University

Tbilisi, , Georgia

Site Status RECRUITING

JSC K. Eristavi National Center of Experimental and Clinical Surgery

Tbilisi, , Georgia

Site Status RECRUITING

LTD Multiprofile Clinic Concilium Medulla

Tbilisi, , Georgia

Site Status RECRUITING

Klinik und Poliklinik fur Kinder- und Jugendmedizin, Universitatsklinikum Carl Gustav Carus an der TU

Dresden, , Germany

Site Status RECRUITING

Universitaetsklinikum Carl Gustav Carus Tu Dresden

Dresden, , Germany

Site Status RECRUITING

Hellenic Airforce 251 General Hospital

Athens, , Greece

Site Status RECRUITING

University General Hospital of Heraklion

Heraklion, , Greece

Site Status RECRUITING

The Olympion Private General Clinic

Pátrai, , Greece

Site Status RECRUITING

AHEPA General Hospital of Thessaloniki

Thessaloniki, , Greece

Site Status RECRUITING

CMC Deli Klinika

Budapest, , Hungary

Site Status RECRUITING

Semmelweis University

Budapest, , Hungary

Site Status RECRUITING

Teaching Hospital Markusovszky

Szombathely, , Hungary

Site Status RECRUITING

Indira Gandhi Institute Of Medical Sciences

Patna, Bihar, India

Site Status RECRUITING

Nirmal Hospital Private Limited

Surat, Gujarat, India

Site Status RECRUITING

Pacific Medical College and Hospital

Udaipur, Rajasthan, India

Site Status RECRUITING

Yashoda Hospital

Hyderabad, Telangana, India

Site Status RECRUITING

Ruth Rappaport Children's Hospital, Rambam Health Corporation

Haifa, , Israel

Site Status RECRUITING

Hadassah University Hospital-ein Kerem

Jerusalem, , Israel

Site Status RECRUITING

Schneider Children's Medical Center of Israel

Petah Tikva, , Israel

Site Status RECRUITING

The Chaim Sheba Medical Center

Ramat Gan, , Israel

Site Status RECRUITING

A. O. U. delle Marche

Torrette, Ancona, Italy

Site Status RECRUITING

ASST Spedali Civili di Brescia

Brescia, , Italy

Site Status RECRUITING

IRCCS Instituto G. Gaslini

Genova, , Italy

Site Status RECRUITING

Istituto Scientifico Romagnolo Per Lo Studio E La Cura Dei Tumori (IRST) Dino Amadori

Meldola, , Italy

Site Status RECRUITING

IRCCS San Gerardo Dei Tintori

Monza, , Italy

Site Status RECRUITING

Bambino Gesu Children's Hospital

Roma, , Italy

Site Status RECRUITING

Hospital Pakar Kanak-Kanak UKM

Kuala Lumpur, , Malaysia

Site Status RECRUITING

Unidade Local de Saúde da Região de Aveiro

Aveiro, , Portugal

Site Status RECRUITING

Unidade Local de Saude Coimbra - Paediatric Hospital

Coimbra, , Portugal

Site Status RECRUITING

Unidade Local de Saude Coimbra - Quinta dos Vales

Coimbra, , Portugal

Site Status RECRUITING

Unidade Local de Saude Santa Maria

Lisbon, , Portugal

Site Status RECRUITING

Spitalul Clinic Colentina Site 1

Bucharest, , Romania

Site Status RECRUITING

Spitalul Clinic Colentina Site 2

Bucharest, , Romania

Site Status RECRUITING

Institutul Clinic Fundeni

Bucharest, , Romania

Site Status RECRUITING

Institutul Clinic Fundeni

Bucharest, , Romania

Site Status RECRUITING

Spitalul Clinic Județean de Urgență

Târgu Mureş, , Romania

Site Status RECRUITING

University Clinical Centre of Serbia

Belgrade, , Serbia

Site Status RECRUITING

ICO Badalona - Institut Català d'Oncologia

Barcelona, , Spain

Site Status RECRUITING

Hospital Del Mar

Barcelona, , Spain

Site Status RECRUITING

Hospital San Pedro De Alcantara

Cáceres, , Spain

Site Status RECRUITING

Hospital Universitario Reina Sofía

Córdoba, , Spain

Site Status WITHDRAWN

Hospital Universitario de Gran Canaria Doctor Negrín

Las Palmas de Gran Canaria, , Spain

Site Status RECRUITING

Hospital De La Princesa

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario Infanta Leonor

Madrid, , Spain

Site Status WITHDRAWN

Hospital Universitario Fundacion Jimenez Diaz

Madrid, , Spain

Site Status RECRUITING

Hospital Universitario Marqués de Valdecilla

Santander, , Spain

Site Status RECRUITING

Hospital Universitario Virgen del Rocio

Seville, , Spain

Site Status RECRUITING

Hospital Universitario y Politecnico de la Fe

Valencia, , Spain

Site Status RECRUITING

Inselspital - University Hospital Bern Site 1

Bern, , Switzerland

Site Status RECRUITING

Inselspital - University Hospital Bern Site 2

Bern, , Switzerland

Site Status RECRUITING

Ramathibodi Hospital

Bangkok, , Thailand

Site Status RECRUITING

Siriraj Hospital

Bangkok, , Thailand

Site Status RECRUITING

Namik Kemal University (Tekirdağ Namık Kemal Üniversitesi)

Tekirdağ, Süleymanpaşa, Turkey (Türkiye)

Site Status RECRUITING

Dr. Abdurrahman Yurtaslan Ankara Oncology Training And Research Hospital

Ankara, , Turkey (Türkiye)

Site Status RECRUITING

Ankara University School of Medicine

Ankara, , Turkey (Türkiye)

Site Status RECRUITING

Uludag University Faculty Of Medicine

Nilufer, , Turkey (Türkiye)

Site Status RECRUITING

Ondokuz Mayis University Medical Faculty Hospital

Samsun, , Turkey (Türkiye)

Site Status RECRUITING

Lviv Regional Clinical Diagnostic Centre

Lviv, Oblast, Ukraine

Site Status RECRUITING

Medical Center Dobrobut-Clinic LLC

Kyiv, , Ukraine

Site Status RECRUITING

Medical Center Ok Clinic of International Institute of Clinical Research LLC

Kyiv, , Ukraine

Site Status RECRUITING

Llc Onto-Med

Kyiv, , Ukraine

Site Status RECRUITING

Communal non-commercial enterprise "Ternopil City Children's Communal Hospital"

Ternopil, , Ukraine

Site Status RECRUITING

Medical Center The Clinic of Hospodarskyy LLC

Ternopil, , Ukraine

Site Status RECRUITING

University Hospital of Wales

Cardiff, , United Kingdom

Site Status RECRUITING

Cardiff And Vale University Health Board

London, , United Kingdom

Site Status RECRUITING

Royal Free London Nhs Foundation Trust Royal Free Hospital

London, , United Kingdom

Site Status RECRUITING

King's College Hospital

London, , United Kingdom

Site Status RECRUITING

Imperial College Healthcare Nhs Trust - Hammersmith Hospital

London, , United Kingdom

Site Status RECRUITING

Great Ormond street Hospital for Children NHS Trust

London, , United Kingdom

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

United States Argentina Australia Canada Colombia Czechia France Georgia Germany Greece Hungary India Israel Italy Malaysia Portugal Romania Serbia Spain Switzerland Thailand Turkey (Türkiye) Ukraine United Kingdom

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Patient Affairs and Advocacy

Role: CONTACT

Phone: 857-529-5779

Email: [email protected]

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

2023-508482-32-00

Identifier Type: CTIS

Identifier Source: secondary_id

X4P-001-110

Identifier Type: -

Identifier Source: org_study_id