{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T14:19:13Z","timestamp":1788185953305,"version":"build-2803163510"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T00:00:00Z","timestamp":1774828800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T00:00:00Z","timestamp":1774828800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100015459","name":"Danish Cancer Society Research Center","doi-asserted-by":"publisher","award":["R352\u2010A20744"],"award-info":[{"award-number":["R352\u2010A20744"]}],"id":[{"id":"10.13039\/100015459","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004836","name":"Danmarks Frie Forskningsfond","doi-asserted-by":"publisher","award":["3101\u201000106B"],"award-info":[{"award-number":["3101\u201000106B"]}],"id":[{"id":"10.13039\/501100004836","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Adult hemophagocytic lymphohistiocytosis (HLH) is a rare, life\u2010threatening syndrome triggered by various conditions. A nationwide study of the incidence and outcomes of HLH in Denmark over 23\u2009years (2000\u20132023) was performed. Adults (\u2265\u00a018\u2009years) with HLH and triggering diseases were identified in the Danish National Patient Registry and\/or the Danish Pathology Registry. A total of 325 cases were identified. The incidence increased from 0.82 per 1 million person\u2010years (1\u2009M\u2010PY) (95% CI 0.61\u20131.04) in 2000\u20132011 to 4.05 per 1\u2009M\u2010PY (95% CI 3.56\u20134.53) in 2012\u20132023. Hematologic malignancies were the triggering diagnosis in 46% of cases, mostly lymphomas (32%). There was no clear improvement in overall survival over time. However, survival differed markedly between subgroups, with the lowest 1\u2010year overall survival of 31% for hematologic malignancy\u2013associated HLH, compared with 75% for idiopathic HLH. In conclusion, HLH remains a rare syndrome with a dismal prognosis, especially when associated with hematologic malignancy.<\/jats:p>","DOI":"10.1111\/ejh.70181","type":"journal-article","created":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T11:08:03Z","timestamp":1774868883000},"page":"210-218","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Incidence and Survival of Hemophagocytic Lymphohistiocytosis Over Two Decades: A Population\u2010Based Study"],"prefix":"10.1111","volume":"117","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3137-3809","authenticated-orcid":false,"given":"Mads Okkels Birk","family":"Lorenzen","sequence":"first","affiliation":[{"name":"Department of Hematology Aarhus University Hospital  Aarhus Denmark"},{"name":"Department of Molecular Medicine Aarhus University Hospital  Aarhus Denmark"},{"name":"Department of Clinical Medicine Aarhus University  Aarhus 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