Sequential Cadaveric Lung and Bone Marrow Transplant for Immune Deficiency Diseases

Exclusion Criteria

• •1. Inability or unwillingness of a participant to give written informed consent or comply with study protocol.
• •2. Patients who have underlying malignant conditions.
• •3. Patients who have non-malignant conditions not requiring hematopoietic stem cell transplantation.
• •4. HIV positive by serology or PCR, HTLV positive by serology.
• •5. Females who are pregnant or who are lactating.
• •6. Allergy to DMSO or any other ingredient used in the manufacturing of the stem cell product.
• •7. Uncontrolled pulmonary infection, as determined by radiographic findings and/or significant clinical deterioration. NOTE: Pulmonary colonization with multiple organisms is common, and will not be considered an exclusion criterion.
• •8. Uncontrolled systemic infection, as determined by the appropriate confirmatory testing e.g. blood cultures, PCR testing, etc.
• •9. Recent recipient of any licensed or investigational live attenuated vaccine(s) within 4 weeks of transplant.
• •10. Past or current medical problems or findings from physical examination or laboratory testing that are not listed above, which, in the opinion of the investigator, may pose additional risks from participation in the study, may interfere with the participant's ability to comply with study requirements or that may impact the quality or interpretation of the data obtained from the study.
• •Eligibility Criteria to proceed to Bone Marrow Transplant
• •1. GFR ≥ 50 mL/min/1.73 m2.
• •2. AST, ALT ≤ 4x upper limit of normal, total bilirubin ≤ 2.5 mg/dL.
• •3. Cardiac ejection fraction ≥ 40% or shortening fraction of at least 26%.
• •4. HIV negative by serology and PCR.
• •5. HTLV serology negative.
• •6. FVC and FEV1 ≥40% predicted for age and SpO2 of \>90% at rest on room air AND with clearance by the lung transplant team.
• •7. Absence of uncontrolled infection as determined by positive blood cultures and radiographic progression of previous sites in particular pulmonary densities during the past 2 weeks prior to chemotherapy.
• •8. Absence of clinically significant Acute Cellular Rejection (A2-A4 and/or B2R rejection).