This is a phase II exploratory study conducted to evaluate the safety and efficacy of the combination of Ibuprofen, G-CSF and Plerixafor as stem cell mobilization regimen in patients affected by X-CGD.

We designed a mobilization trial with the aim of collecting a sufficient number of HSPC in X-CGD patients; it is well known that this procedure is challenging for these patients, potentially due to functional defects induced by their chronic inflammatory state.

The combination of G-CSF and Plerixafor is considered state of the art for HSPC harvest in gene therapy trials; we considered to add a non-steroidal inflammatory drug to increase HSPC mobilization and reduce inflammation that could have a role in altering HSPC content.

If this trial confirms the synergistic effect of the three drugs under investigation, such a regimen will be considered for a HSPC mobilization in future gene therapy trial for X-CGD patients.

Inclusion Criteria:

• Genetic diagnosis of X-CGD
• 18-45 years of age
• Karnofsky Index > 80 %
• Adequate cardiac, renal, hepatic and pulmonary function.
• Negative thrombophilic screen and negative history for previous thrombotic events
• Written informed consent

Exclusion Criteria:

• Previous Bone Marrow Transplantation or previous Gene Therapy.
• Use of other investigational agents within 4 weeks prior to study enrolment (within 6 weeks if use of long-acting agents).
• Ongoing IFN-γ treatment (within 4 weeks).
• Symptomatic inflammatory bowel disease.
• Symptomatic viral, bacterial, or fungal infection within 6 weeks of eligibility
• Neoplasia (except local skin cancer) or history of "familial" cancer
• Myelodysplasia or other serious hematological disorder
• History of uncontrolled seizures and deep venous thrombosis
• Other systemic disease judged as incompatible with the procedure
• Positivity for HIV and/or HCV RNA and/or HbsAg and/or HBV DNA
• Active alcohol or substance abuse within 6 months of the study.
• Contraindications to IBU, G-CSF, Plerixafor or Pantoprazole administration