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

Key Inclusion Criteria:

• Diagnosis of congenital or acquired primary autoimmune and idiopathic chronicneutropenic disorder ≥6 months prior to the screening visit that is not attributableto 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), Kostmanndisease

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 thatmay be eligible for enrollment can be clarified and approved upon discussionwith study Medical Monitor and Sponsor.

• Have an ANC <1000 cells/µL during screening (single ANC value from hematology) andconfirmed trough mean ANC (mean value of multiple ANC measurements over 6 hours) atbaseline visit, with no clinical evidence of systemic infection.

• Prior history of recurrent and/or serious infections during the 12 months precedingthe screening visit (that is, suffering sequelae of chronic neutropenia), as definedby having at least 2 infections in the last 12 months that meet the followingcriteria:

• Infection requiring the use of antibiotics (intravenous [IV]/oral); OR

• Infection requiring a visit to healthcare facility (including but not limited toemergency room visit, urgent care facility, primary care physician's office, orin-patient hospitalization);

AND for all potential participants:

• Infections considered by the Investigator to be likely related to the potentialparticipant's CN disorder.

• Participants who are on G-CSF or other active background therapy must have beenreceiving these therapies during the previous 12 months while continuing to sufferfrom infections, be on a stable dose and dosing schedule for ≥4 weeks prior toscreening 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 therapydoses/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, rheumatoidarthritis, inflammatory bowel disease, graft-versus-host disease, thyroiddisease

5. Nutritional deficiency, for example, vitamin B12, folic acid, copper, caloricmalnutrition

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:

4. Isolated with a cyclic presentation, for example, elastase, neutrophilexpressed (ELANE)

5. Associated with immune dysregulation, for example, common variableimmunodeficiency (CVID), autoimmune lymphoproliferative syndrome (ALPS),familial hemophagocytic lymphohistiocytosis, Chédiak-Higashi syndrome,GATA-binding protein 2 (GATA2) deficiency syndrome

6. Associated with bone marrow failure, for example, Fanconi anemia,Diamond-Blackfan anemia

7. Neutropenia associated with a Duffy-null phenotype (formerly known as benignethnic neutropenia). However, a participant with an autosomal dominantpathogenic variant in a gene associated with CN on a Duffy-null background maybe eligible for inclusion

• A medical or personal condition that may potentially compromise the safety of theparticipant, may preclude the participant's successful completion of the clinicalstudy, or could, in the opinion of the Investigator or the Sponsor, interfere withthe 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) inthe past 6 months.

• Participants taking pegylated-G-CSF unless they have a diagnosis of congenitalneutropenia confirmed at screening.

• Participant is currently taking or has taken other investigational drug <30 daysprior to the screening visit or 5 half-lives, whichever is longer.

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