Table 1.
Primary immunodeficiencies that are associated with loss of immune control by innate lymphocytes and EBV-associated pathologies.
| Affected protein | EBV-associated pathology | Affected innate lymphocytes | Reference |
|---|---|---|---|
| Cytotoxic machinery | |||
| Perforin | CAEBV, HLH | NK, NKT, γδT | (46) |
| Munc13-4 | CAEBV, HLH | NK, NKT, γδT | (47) |
| Munc18-2 | CAEBV, HLH | NK, NKT, γδT | (48) |
| DNA-binding proteins | |||
| GATA2 | CAEBV, HLH | NK | (18, 45) |
| MCM4 | EBV lymphoma | NK | (75, 76) |
| NF-κB1 | EBV lymphoma | NK | (73, 74) |
| Costimulatory receptors and their ligands | |||
| CD27 | EBV lymphoma | NKT | (66–68) |
| CD70 | EBV-positive Hodgkin’s lymphoma | NKT | (64, 65) |
| CD16 | EBV-positive Castleman’s disease | NK | (77, 78) |
| NKG2D and TCR (because of MagT1 deficiency) | EBV lymphoma | NK, γδT | (69–72) |
| Signaling molecules | |||
| SAP | EBV lymphoma, IM, HLH | NKT | (49–51, 54, 55) |
| ITK | EBV lymphoma | NKT | (56–63) |
| RasGRP1 | EBV lymphoma | NKT | (79) |
| PI3K 110δ | EBV viremia | NK | (82) |
| Others | |||
| XIAP | IM, HLH | NKT | (52, 53) |
| Coronin 1A | EBV lymphoma | NKT | (80) |
| CTP synthase 1 | IM, EBV lymphoma | NKT | (81) |
CAEBV, chronic active EBV; HLH, hemophagocytic lymphohistiocytosis; IM, infectious mononucleosis; NK, natural killer; EBV, Epstein–Barr virus; NKT, natural killer T; SAP, SLAM-associated protein; XIAP, X-linked inhibitor of apoptosis; ITK, inducible T cell kinase; MCM4, minichromosome maintenance complex component 4; PI3K, phosphatidylinositol-3-kinase.