{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T22:19:58Z","timestamp":1792793998001,"version":"4.5.0"},"reference-count":50,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,6,24]],"date-time":"2021-06-24T00:00:00Z","timestamp":1624492800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Epstein\u2013Barr virus (EBV) is a ubiquitous herpesvirus with rare but severe potential for lymphoproliferative complications. EBV is associated with a variety of presentations of haemophagocytic lymphohistiocytosis (HLH). HLH is a life\u2010threatening hyperinflammatory syndrome that can occur in patients with genetic defects associated with dysregulation of the immune response (familial HLH) or arise in patients with underlying infection or malignancy (non\u2010familial or secondary HLH). EBV can both serve as the incidental trigger of familial HLH or as the driving factor in patients with selective inherited vulnerability (e.g. X\u2010linked lymphoproliferative disease). Alternatively, acute infection can idiosyncratically cause non\u2010neoplastic HLH in patients without inherited predisposition (i.e. secondary HLH), while EBV\u2010associated T\/natural killer (NK)\u2010cell lymphoproliferative disorders and lymphomas can cause neoplasia\u2010associated HLH. The present review will discern between EBV\u2010associated familial and non\u2010familial HLH and highlight diagnostic and therapeutic considerations. Non\u2010familial EBV\u2010associated HLH is a major diagnostic dilemma, as it represents a diverse spectrum of disease ranging from highly curable (non\u2010neoplastic EBV\u2010HLH) to indolent but incurable (chronic active EBV) to acutely fatal (systemic EBV\u2010positive T\u2010cell lymphoma of childhood). Increased clinical awareness and understanding of this rare and potentially devastating subset of EBV\u2010related complications is desperately needed to improve survival for patients with neoplasia\u2010associated HLH.<\/jats:p>","DOI":"10.1111\/bjh.17638","type":"journal-article","created":{"date-parts":[[2021,6,24]],"date-time":"2021-06-24T23:09:03Z","timestamp":1624576143000},"page":"31-44","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":124,"title":["Haemophagocytic lymphohistiocytosis and Epstein\u2013Barr virus: a complex relationship with diverse origins, expression and outcomes"],"prefix":"10.1111","volume":"196","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6115-1046","authenticated-orcid":false,"given":"Nader Kim","family":"El\u2010Mallawany","sequence":"first","affiliation":[{"name":"Department of Paediatrics Baylor College of Medicine Houston TX USA"},{"name":"Texas Children\u2019s Cancer and Haematology 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