• INCLUSION CRITERIA:

NORMAL VOLUNTEERS:

Healthy adults aged 18-65 without active current infection or history of recurrent infection weighing greater than 50kg.

For purposes of directed allogeneic donation only, eligibility will be extended to include healthy subjects weighing 12 or more kilograms without active infection or history of recurrent infections.

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).

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.

Willingness to allow blood cell samples to be stored.

Normal volunteers who participate in this protocol will be assigned to one of 2 groups. The determination of group assignment depends on what we intend to do with the cells we collect from the volunteer. The groups will be designated as Healthy Volunteers or Healthy Donors for purposes of clarity.

PATIENTS:

CGD patients must have a demonstrated specific cellular defect in oxidant production by phagocytic cells and history of recurrent infections compatible with a diagnosis of CGD. Specifically, patients must have had studies at NIH confirming abnormal oxidase activity consistent with a diagnosis of CGD.

LAD patients must have a FACS analysis demonstrating deficiency of leukocyte integrins (CD11a,b,c/CD18).

X-SCID patients must have a documented abnormality or deficiency of the common gamma chain of the IL2 receptor or defects in numbers and function of B lymphocyte and/or T lymphocytes consistent with XSCID.

IGR deficiency patients must demonstrate an absence or deficiency of this receptor as assessed by flow cytometry or Western immunoblot, or a defined mutation in the interferon gamma receptor by sequencing analysis, or a defect in function of the receptor for interferon gamma as evidenced by a lack or deficiency of stat-1 protein phosphorylation in response to stimulation of monocytes with interferon gamma.

Other patients may be enrolled who have a history of recurrent infection where an inherited phagocytic cell defect might be the underlying abnormality.

Some patients may have active bacterial or fungal infection at the time of study entry.

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).

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.

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).

Willingness to allow blood cell samples to be stored.

EXCLUSION CRITERIA:

NORMAL VOLUNTEERS AND DONORS:

Active bacterial, fungal or viral infection as evidenced by history, physical exam (temperature greater than 38 degrees Celsius) or WBC greater than 9000 are excluded.

Females who are pregnant or lactating as determined by history and/or pregnancy test are excluded.

History of vasculitis or similar disorder.

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 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).

Volunteers without peripheral venous access in arm veins adequate for apheresis.

Weight under 12 kilograms.

PATIENTS:

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.

Female patients who are pregnant or lactating as determined by history and/or positive pregnancy test are excluded.

History of vasculitis or similar disorder.

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.

Weight under 12 kilograms.

Donors or Patients being considered for bone marrow harvesting:

Who are unable to lie prone during the bone marrow harvesting procedure.

Who are unable to tolerate general anesthesia during the bone marrow harvesting procedure.