Table 2.

Combined Immunodeficiencies in the Differential Diagnosis of ZAP70-Related CID

DisorderGene InvolvedMode of
Inheritance		Lymphocyte Phenotype
TBNKOther
ZAP70-related CID ZAP70 AR+++CD4+/CD8-
Familial CD8 deficiency CD8A AR+++CD4+/CD8–
CD25 deficiency IL2RA AR+++CD4–/CD8+
MHC II deficiency (BLS)See Major histocompatibility complex class II deficiency.AR+++CD4–/CD8+

BLS = bare lymphocyte syndrome; MHC II = major histocompatibility complex class II

From: ZAP70-Related Combined Immunodeficiency

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