Allogeneic Hematopoietic Stem Cell Transplantation for Chronic Granulomatous Disease (CGD) With an Alemtuzumab, Busulfan and TBI-based Conditioning Regimen Combined With Cytokine (IL-6, +/- IFN-gamma) Antagonists

Eligibility Criteria

• * Must have the ability to comprehend and a willingness to sign the informed consent. For pediatric patients, must have a parent/guardian who can sign consent if the donor is a minor; assent will be obtained from minors as appropriate.
• * Must have confirmed diagnosis of CGD.
• * Must have sufficient complications from underlying disease to warrant undergoing transplantation (either a history of or ongoing inflammation/CGD-related autoimmunity OR a CGD-related infection while on prophylaxis) OR have a Quartile 1 or 2 residual oxidase production level.
• * Ages 4 years-65 years.
• * HLA-matched family donor graft or an HLA-matched unrelated PBSC graft (10/10 or 9/10 mismatch) available.
• * Must be human immunodeficiency virus (HIV) negative.
• * When discharged from the hospital the participant must be able to stay within 1 hour s travel of the NIH for the first 3 months after transplantation.
• * Must have a family member or other designated care provider to assist with care during the post-transplant period when the patient is in the outpatient setting.
• * Must provide a durable power of attorney for health care decisions to an appropriate adult relative or guardian in accordance with NIH 200 'NIH Durable Power of Attorney for Health Care Decision Making.'
• * Females of child-bearing potential must agree to consistently use one form of contraception from 1 month prior to study entry and for at least 1 year post transplant. Male participants must agree to consistently use contraception for 1 year post transplant. Acceptable forms of contraception are:
• * Contraceptive pills or patch, Norplant \[Registered\], Depo-Provera \[Registered\], or other FDA-approved contraceptive method.
• * Male partner has previously undergone a vasectomy.
• * Male participants will be advised to consistently use contraception throughout study participation and for 3 months post-transplant.
• * Stated willingness to comply with all study procedures and is available for protocol visits for the duration of the study when possible.
• * Patients who have a CRP of greater than 100 but otherwise meet inclusion criteria will be enrolled on the high-risk arm.
• * CRP will be assessed no more than 7 weeks and no less than 6 weeks prior to anticipated transplant to determine on which arm the patient will be treated.
• * Ejection fraction of less than 30% by echocardiography.
• * Forced expiratory volume (FEV1%) of less than 35% and/or an adjusted diffusing capacity of lung of carbon monoxide (adj DLCO) of less than 30%.
• * Transaminases \>5x upper limit of normal based on the individual s clinical situation and at the discretion of the investigator.
• * Psychiatric disorder or mental deficiency severe enough as to make compliance with the HSCT treatment unlikely, and/or to make regulatorily and legally effective informed consent impossible.
• * Major anticipated illness or organ failure incompatible with survival from allogeneic peripheral blood stem cell (AlloPBSC) transplant.
• * Pregnant or lactating.
• * Uncontrolled seizure disorder per principal investigator (PI) discretion.
• * Individuals older than 65 years are excluded. It is known from standard transplantation that these individuals have a higher risk of morbidity and mortality related to transplantation. Given the investigational nature of this protocol, the risk-benefit ratio is not warranted to include these individuals at this time.
• * Active TB infection.
• * Any condition or circumstance that the PI feels would create difficulty in maintaining compliance with the requirements of this protocol.
• * Individuals who are not willing to submit their information as part of the alemtuzumab (Campath \[Registered\]) Distribution Program application or participants whom the Distribution Program committee has determined are not qualified to receive alemtuzumab.