Table 2.
Primary immunodeficiencies and the outcome of herpesviral infections.
| Host molecules | Virus involved | Outcome | References |
|---|---|---|---|
| PRF1, STXBP2 | EBV | Inefficient immune response and leads to the familial HLHs | Reviewed in Parvaneh et al. (2013) |
| ITK | EBV | B-cell lymphoproliferation, Hodgkin's lymphoma, hepatosplenomegaly, cytopenia, hypogammaglobulinaemia, and high viral load in host | Huck et al., 2009; Stepensky et al., 2011; Linka et al., 2012 |
| CD27 | EBV | Hypogammaglobulinaemia, reduced memory B cell count, diminished CD8+ T cell and iNKT cell function | van Montfrans et al., 2012; Salzer et al., 2013 |
| MAGT1 | EBV | Abrogates T cell activation and reduced CD4+ T cell count | Li et al., 2011 |
| STK4 | EBV | Hypergammaglobulinaemia, reduced TCR repertoire, and naive T cell count, enhanced recurrent infection | Abdollahpour et al., 2012; Nehme et al., 2012 |
| CORO1A | EBV | B-cell lymphoproliferation | Moshous et al., 2013 |
| SH2D1A | EBV | HLH | Marsh and Filipovich, 2011 |
| MCM4 | EBV | EBV lymphoma | Reviewed in Ruffner et al. (2017) |
| OX40 | KSHV | Enhanced susceptibility to mycobacteria | |
| IFNGR1 | KSHV, VZV | Inadequate cytokine signaling | |
| DOCK8, NEMO, GATA2, STAT1 GOF, STK4, WHIM | HSV, VZV | Chronic infection, elevated cutaneous coinfection | |
| STAT3 LOF | VZV | Altered T cell population leading to immunopathological response | |
| CXCR4 | HSV | Abnormal neutrophils, pancytopenia | |
| WAS | HSV | Eczema, thrombocytopenia | |
| UNC-93B | HSV | Impaired type I and type II IFN responses, HSE | Casrouge et al., 2006 |
| TLR3 | HSV | Impaired type I and type II IFN responses, HSE | Casrouge et al., 2006 |
| PRF1 | CMV | Diminished CD8+ T cell and NK cell mediated killing | Kägi et al., 1996 |
| UNC13D | CMV | Familial HLH | Crozat et al., 2007 |
PRF1, Perforin 1; STXBP2, Syntaxin binding protein 2; ITK, interleukin-2 inducible T cell kinase; EBV, Epstein-Barr virus; MAGT1, magnesium transporter 1; STK4, serine/threonine protein kinase 4; CORO1A, Coronin-1A; SH2D1A, Src homology 2 domain-containing gene 1 A; HLH, hemophagocytic lymphohistiocytosis; XIAP, X-linked inhibitor of apoptosis protein; MCM4, minichromosome maintenance protein 4; iNKT, invariant natural killer T cell; KSHV, kaposi sarcoma herpes virus; IFNGR1, interferon gamma receptor 1; DOCK8, dedicator of cytokinesis 8; NEMO, NF-κ B essential modulator; STAT1, signal transducer and activator of transcription 1; GOF, gain of function; LOF, loss of function; STK4, serine/threonine protein kinase 4; WHIM, warts, hypogammaglobulinemia, infections, and myelokathexis; VZV, varicella zoster virus, CMV, cytomegalovirus, CXCR4, C-X-C chemokine receptor 4; WAS, Wiskott-Aldrich syndrome; HSE, herpes simplex encephalitis; TLR3, toll like receptor 3.