We classified patients with a hypomorphic mutation in NHEJ components (RAG1 hypomorphic with 48% residual activity [36], DCLRE1C- OMIM #602450) and a patient with mutation in JAK3 (OMIM #600802) [37] as atypical SCID, due to incompatibility with the standard diagnostic criteria of the European Society for Immunodeficiencies (ESID) and recent phenotypic classification of the International Union of Immunological Societies (IUIS) [38]. Here, JAK3 is linked to immunodeficiency disease.