A significant proportion of patients (45%) harbored germline variants in genes associated with inborn errors of immunity (IEI), such as AIRE, CFH, CFHR5, CR2, C2, C9, MASP1, LIG1, TNFRSF13B, and BLNK. Germline variants affecting complement genes were more prevalent in MDS-h patients than in AA patients (54% vs. 17%, p < 0.05). Here, LIG1 is linked to inborn error of immunity.