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Epstein\u2013Barr virus (EBV), a human herpesvirus, has long been of interest because of its complex interaction with the immune system. Thus, it causes both infectious mononucleosis (IM), an immunopathologic disease associated with exaggerated host responses, and at least one malignancy, EBV\u2010positive lymphoproliferative disease, when those responses are impaired. Here, we describe the full range of PIDs currently linked with an increased risk of EBV\u2010associated disease. These provide examples where IM\u2010like immunopathology is fatally exaggerated, and others where responses impaired at the stage of induction, expansion, or effector function predispose to malignancy. Current evidence from this rapidly moving field supports the view that lesions in both natural killer cell and T\u00a0cell function can lead to EBV pathology.<\/jats:p>","DOI":"10.1111\/nyas.12937","type":"journal-article","created":{"date-parts":[[2015,9,28]],"date-time":"2015-09-28T19:12:55Z","timestamp":1443467575000},"page":"22-44","source":"Crossref","is-referenced-by-count":53,"title":["Primary immunodeficiencies and the control of Epstein\u2013Barr virus infection"],"prefix":"10.1111","volume":"1356","author":[{"given":"Umaimainthan","family":"Palendira","sequence":"first","affiliation":[{"name":"Centenary Institute  Newtown New South Wales Australia"},{"name":"Discipline of Medicine, Sydney Medical School University of Sydney  NSW Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan B.","family":"Rickinson","sequence":"additional","affiliation":[{"name":"Cancer Sciences and Centre for Human Virology University of Birmingham  Birmingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,9,28]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.immuni.2007.11.012"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri1264"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/cid\/ciu646"},{"key":"e_1_2_12_5_1","volume-title":"Fields Virology","author":"Longnecker R.M.","year":"2013"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrc1452"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-immunol-032414-112326"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.it.2014.01.003"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra032015"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.coviro.2013.04.005"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/emm.2014.84"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-7613(00)00048-0"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1038\/35095584"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/nri1201"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1002697"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/infdis\/jis646"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20011692"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20041542"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1084\/jem.20022058"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1100590"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-01-553024"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2013.11.041"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.01777-06"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-01-480665"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-09-0828"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/cmi.2011.66"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2014.07.026"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12253-010-9329-8"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2013.96"},{"key":"e_1_2_12_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcancer.2014.04.004"},{"key":"e_1_2_12_31_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrclinonc.2012.111"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-12-063008"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2006-10-048173"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2336091100"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0409872102"},{"key":"e_1_2_12_36_1","doi-asserted-by":"publisher","DOI":"10.1097\/CCO.0b013e328355e115"},{"key":"e_1_2_12_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.beha.2012.01.005"},{"key":"e_1_2_12_38_1","first-page":"1","article-title":"Primary immunodeficiency diseases: an update on the classification from the International Union of immunological societies expert committee for primary immunodeficiency","volume":"5","author":"Al\u2010Herz W.","year":"2014","journal-title":"Front. 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