Table 2.
Primary immunodeficiencies that predispose for KSHV-associated diseases.
| Affected protein ‘name of syndrome’ | KSHV-associated disease | Innate immune system changes | Adaptive immune system changes | References |
|---|---|---|---|---|
| Th1 effector function | ||||
| IFNγR1 | KS | – | CD4+ T cell lymphopenia | Camcioglu et al.2004 |
| STAT4 | KS | – | Decreased Th1 differentiation | Aavikko et al.2015 |
| TCR signaling | ||||
| STIM1 | KS | – | Deficient T cell activation due to compromised Ca2+ influx | Byun et al.2010 |
| WASP ‘Wiskott Aldrich syndrome’ | KS | – | CD4+ T cell lymphopenia, deficiency in immunological synapse formation | Picard et al.2006 |
| Co-stimulation | ||||
| OX40 | KS | – | Impaired effector memory T cell populations | Byun et al.2013 |
| MagT1 ‘XMEN’ | KS | Decreased NK cell maturation and NKG2D expression | Compromised T cell receptor signaling | Brigida et al.2017 |