Table 1: Primary immune Deficiencies and their genetic background, associated with T-cell dysfunction and aberrant IgE production.

Primary immune deficiencies with elevated IgE
T-cell dysfunctionImmune deficiencyGenetic background

Treg cell deficiencyImmune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX)Forkhead box protein 3 (Foxp3) signal transducer and activator of transcription 5b (STAT5b)CD25
Treg cell dysfunctionWiskott-Aldrich syndrome (WAS)Wiskott-Aldrich syndrome protein (WASP)
Treg cell deficiency T-cell oligoclonalityOmenn syndrome (OS)recombination activation genes (RAG1,RAG2) Artemis IL-7R zeta-associated protein, 70 kD (ZAP-70,) DNA ligase
Reduced NK cell cytotoxicityskewed Th1 phenotypeComel-Netherton syndrome (CNS)Serin protease inhibitor Kazal type (SPINK) lymphoepithelial Kazal type inhibitor (LEKTI)
T-cell oligoclonalityDiGeorge syndrome (DGS)—atypical complete formMicrodeletion 22q11
Reduced Th17 cells Treg cell dysfunctionmultiple cytokine signaling defectHyperimmunoglobulin E syndrome (HIES)Signal transducer and activator of transcription 3 (STAT3) dedicator of cytokinesis 8 (DOCK8) tyrosine kinase 2 (Tyk2)