Regenerative Medicine to Restore Hematopoiesis and Immune Function in Immunodeficiencies and Inherited Bone Marrow Failures

Inclusion Criteria Cohort A: Primary Immune Deficiencies with indication for HCT: * Chronic granulomatous disease (CGD) * Wiskott-Aldrich syndrome (WAS) * Hyper-IgM syndrome * Common variable immunodeficiency (CVID) * Leukocyte adhesion deficiency-1 (LAD-1) * Severe Combined Immunodeficiency (SCID) * CTLA-4 deficiency * CARD9 deficiency * DOCK8 deficiency Immune Dysregulatory Syndromes: * Immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome * Hemophagocytic lymphohistiocytosis (HLH) or related disorder with indication for transplant * CAEBV: Patients with chronic EBV infection (CAEBV) with indication for BMT: Inherited Bone marrow failure disorders * Congenital amegakaryocytic thrombocytopenia (CAMT) * Diamond Blackfan anemia (DBA) * Shwachman Diamond Syndrome (SDS) * Thrombocytopenia Absent Radii (TAR) * Glanzmans thrombasthenia (GT) * Kostmann syndrome * Other indications and/or other PID, IDS, and IBMFS diagnoses as deemed appropriate by the PI. Cohort B: Short telomere syndrome Cohort C: Confirmed diagnosis of Fanconi anemia or non-Fanconi DNA-dsb repair disorders * Fanconi anemia * Non-Fanconi DNA-dsb repair disorders * Cerunnos-XRCC4-like factor deficiency (XLF or NHEJ1) * DNA ligase IV deficiency (LIG4) * Nijmegen breakage syndrome (NBS) * Increased DNA breakage after exposure of patient cells to DNA cross-linking agents such as diepoxybutane or mitomycin C and germline mutation(s) in an identified Fanconi pathway gene. Available donor…