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In the circulation, <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content> primarily infects the B cells, and protective immunity is mediated by <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010specific cytotoxic T cells (<jats:styled-content style=\"fixed-case\">CTL<\/jats:styled-content>s) and natural killer (<jats:styled-content style=\"fixed-case\">NK<\/jats:styled-content>) cells. However, <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content> has been linked to several devastating diseases, such as haemophagocytic lymphohistiocytosis (<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>) and lymphoproliferative diseases in the immunocompromised host. Some types of primary immunodeficiencies (<jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s) are characterized by the development of <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010associated complications as their predominant clinical feature. The study of such genetic diseases presents an ideal opportunity for a better understanding of the biology of the immune responses against <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>. Here, we summarize the range of <jats:styled-content style=\"fixed-case\">PID<\/jats:styled-content>s that are predisposed to <jats:styled-content style=\"fixed-case\">EBV<\/jats:styled-content>\u2010associated haematological diseases, describing their clinical picture and pathogenetic mechanisms.<\/jats:p>","DOI":"10.1111\/bjh.12422","type":"journal-article","created":{"date-parts":[[2013,6,11]],"date-time":"2013-06-11T12:38:53Z","timestamp":1370954333000},"page":"573-586","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":93,"title":["Primary immunodeficiencies predisposed to Epstein\u2010Barr virus\u2010driven haematological diseases"],"prefix":"10.1111","volume":"162","author":[{"given":"Nima","family":"Parvaneh","sequence":"first","affiliation":[{"name":"Paediatric Infectious Diseases Research Centre Children's Medical Centre Tehran University of Medical Sciences Tehran Iran"},{"name":"Division of Allergy and Clinical 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