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. Author manuscript; available in PMC: 2018 Mar 1.
Published in final edited form as: Biol Blood Marrow Transplant. 2017 Jan 6;23(3):379–387. doi: 10.1016/j.bbmt.2016.12.619

TABLE 2.

Known data regarding late effect risks in specific genotypes and the impact on alkylator therapy on those risks

SCID Genotype Late Effects Compounded by Conditioning Use (Busulfan/Alkylator) Late Effect Risks Lowered by Conditioning Use (Busulfan/Alkylator) No Known Conditioning effect on Late Complication
IL2-RG, JAK3 IVIG dependence (longterm) Warts
IL-7R, CD3, CD45 Warts (IL-7R)
RAG1/RAG2 Durability of T cell reconstitution;
IVIG dependence (longterm)
Artemis/Other radiosensitive SCID
*With regard to radiosensitive SCID (RS) genotypes: PRKDC, LIG4, NHEJ1, and NBS1 patients may be even more sensitive to alkylators than ART-SCID patients		 Fertility Concerns,
Skeletal Growth,
Dental Complications (delayed/absent eruption, abnormal shape, abnormal enamel)
Hypothyroidism
For RS-SCID, poorer survival with MAC vs RIC		 Durability of T cell reconstitution;
IVIG dependence (longterm)
ADA Decreased survival associated with intensity of conditioning Neurocognitive Impairment (learning challenges, ADHD)
Hearing Impariment
Others Hearing impairment (AK2/Reticular Dysgenesis)