• ELIGIBILITY CRITERIA:

Healthy Volunteers:

1. Healthy adults aged 18-65 without active current infection or history of recurrent infection,
2. Weighs at least 50kg.
3. Normal renal function (creatinine less than or equal to 1.5 mg/dL; less than or equal to 1+ proteinuria); normal hepatic function (bilirubin less than or equal to 1.5 mg/dL); normal hematologic function (WBC greater than or equal to 2500/mm(3); granulocytes greater than or equal to 1200/mm(3); platelets greater than or equal to 120,000; hematocrit greater than or equal to 38).
4. Normal female volunteers of childbearing potential may be entered if using effective contraception and having a negative serum pregnancy test within one week of beginning G-CSF administration.
5. Willingness to allow blood cell samples to be stored
6. Willingness to allow blood and/or bone marrow samples to be modified to iPS cells

Patients (Patients with a genetically defined PID or clinical history consistent with PID):

1. Patients will have a genetically defined PID or have a clinical history of recurrent infections or other problems suggestive of PID, must be 2-50 years of age,
2. Is at least 10 kilograms body weight (or at least 12 kilograms body weight to have apheresis collection if the sole purpose of the collection is laboratory research).
3. Some patients may have active bacterial or fungal infection at the time of study entry.
4. Preserved renal function (creatinine less than or equal to 2.5 mg/dL; less than or equal to 3+ proteinuria); preserved hepatic function (bilirubin less than or equal to 2.0 mg/dl); preserved hematologic function (WBC greater than or equal to 1000/mm(3); granulocytes greater than or equal to 500/mm(3); platelets greater than or equal to 100,000; hematocrit greater than or equal to 25). For those patients with chronic thrombocytopenia for which the collection is intended for clinical treatment (gene therapy or as rescue stem cells in the context of allogeneic stem cell transplant), patients may participate regardless of platelet count, but must have a platelet count > 40,000 on the day of first apheresis following any required platelet transfusion needed to achieve that level.
5. Patients of childbearing potential may be entered if using effective contraception and having a negative serum pregnancy test within one week of beginning G-CSF administration.
6. All patients will be maintained on their normal regimen of prophylactic medications (i.e. prophylactic antibiotics for all patients, interferon gamma or prednisone for CGD, IVIG for XSCID patients).
7. Willingness to allow blood cell samples to be stored
8. Willingness to allow blood and/or bone marrow samples to be modified to iPS cells

Healthy Donors (HLA matched sibling or other related donor of a patient with PID in need of an allogeneic hematopoietic stem cell transplant):

1. Healthy sibling or other relative of a patient with PID in need of an allogeneic transplant, between 2-65 years of age,
2. Is fully or closely HLA tissue matched to the patient with PID that the hematopoietic stem cell PBSC or bone marrow collection is designated to clinically benefit,
3. Is at least 10 kilograms body weight.
4. Normal renal function (creatinine less than or equal to 1.5 mg/dL; less than or equal to 1+ proteinuria); normal hepatic function (bilirubin less than or equal to 1.5 mg/dL); normal hematologic function (WBC greater than or equal to 2500/mm(3); granulocytes greater than or equal to 1200/mm(3); platelets greater than or equal to 120,000; hematocrit greater than or equal to 38).
5. Normal female donors of childbearing potential may be entered if using effective contraception and having a negative serum pregnancy test within one week of beginning G-CSF administration.
6. Willingness to allow blood cell samples to be stored
7. Willingness to allow blood and/or bone marrow samples to be modified to iPS cells

EXCLUSIONS:

Healthy Volunteers and Healthy Donors:

1. Active bacterial, fungal or viral infection as evidenced by history, physical exam (temperature > 38 degrees C), or WBC > 9000 are excluded.
2. Females who are pregnant or lactating as determined by history and/or pregnancy test are excluded.
3. History of vasculitis or similar disorder.
4. Must be negative by routine blood donor eligibility testing criteria including tests for syphilis (RPR) and TTV Recipient Transplant Panel (includes hepatitis B and C, HIV and HTLV, T. cruzi) (These are routine tests for blood bank donors.)
5. Someone without peripheral venous access in arm veins adequate for apheresis.
6. If in the opinion of the investigator participation in this study places the healthy volunteer or donor at undue risk.

Patients:

1. Patients who are hemodynamically unstable (systolic or diastolic blood pressure fall of 20 mm Hg from the stable patient's baseline measurement) or requiring mechanical respiratory assistance are excluded.
2. Female patients who are pregnant or lactating as determined by history and/or positive pregnancy test are excluded.
3. History of vasculitis or similar disorder.
4. Must be negative by routine blood donor eligibility testing criteria including tests for syphilis (RPR) and TTV Donor Transplant Panel testing (includes hepatitis B and C, HIV and HTLV, T. cruzi). XSCID patients do not make antibodies and false positives may occur because they receive periodic infusions of pooled donations of IVIG. We have observed positive anti-HBc testing in these patients. If this occurs, more specific DNA or antigen testing will be done and must be negative.

Patients or Healthy Donors being considered for clinical scale bone marrow harvesting:

1. Who are unable to lie prone during the bone marrow harvesting procedure.
2. Who are unable to tolerate general anesthesia during the bone marrow harvesting procedure.