∙ Cohort A: Confirmed diagnosis of:

• 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 as follows:

• Fully HLA matched sibling or other first-degree family member.

• Fully HLA matched unrelated 10/10 donor using high-resolution DNA-based typing at the following genetic loci: HLA-A, -B, -C, DRB1, and DQB1.

• Mismatched unrelated donor at 8 or 9/10 alleles, using high-resolution typing as above.

• HLA-haploidentical family members of any degree who match at least one allele of each of the following genetic loci: HLA-A, -B, -C, DRB1, and DQB1. A minimum match of 5/10 is therefore required, and will be considered sufficient evidence that the donor and recipient share one HLA haplotype.

• The patient and/or legal guardian must sign informed consent for BMT.

• Patients with adequate organ function as measured by

• Cardiac: Left ventricular ejection fraction (LVEF) at rest must be ≥ 35%. For patients aged \<13 years, shortening fraction (SF) \> 25% by echocardiogram or LVEF by MUGA may be used.

• Hepatic: Bilirubin ≤ 3.0 mg/dL; and ALT, AST, and Alkaline Phosphatase \< 5 x ULN.

• Renal: Serum creatinine within normal range for age, or if serum creatinine outside normal range for age, then renal function (creatinine clearance or GFR) \> 40 mL/min/1.73m2.

• Pulmonary: PFT with FEV1 and FVC \>/= 50% of normal and DLCO corrected for Hgb \>/= 40% of normal. Patients unable to undergo PFTs should have stable resp status with SaO2 \>90% on a max of 2L/min supplemental O2.

• Karnofsky or Lansky performance status ≥70%

• Females and males of childbearing potential must agree to practice 2 effective methods of contraception at the same time, or agree to abstinence.