{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T05:45:55Z","timestamp":1793857555290,"version":"4.6.1"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2023,5,26]],"date-time":"2023-05-26T00:00:00Z","timestamp":1685059200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,5,26]],"date-time":"2023-05-26T00:00:00Z","timestamp":1685059200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Histio UK"},{"name":"UK Department of Health and Social Care"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Hematol Oncol"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Haemophagocytic lymphohistiocytosis (HLH) is a lethal syndrome of excessive immune activation. We undertook a nationwide study in England of all cases of HLH diagnosed between 2003 and 2018, using linked electronic health data from hospital admissions and death certification. We modelled interactions between demographics and comorbidities and estimated one-year survival by calendar year, age group, gender and comorbidity (haematological malignancy, auto-immune, other malignancy) using Cox regression. There were 1628 people with HLH identified. Overall, crude one-year survival was 50% (95% Confidence interval 48\u201353%) which varied substantially with age (0\u20134: 61%; 5\u201314: 76%; 15\u201354: 61%; &gt;\u200955: 24% <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01), sex (males, 46%, worse than females, 55% <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01) and associated comorbidity (auto-immune, 69%, haematological malignancy 28%, any other malignancy, 37% <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.01). Those aged &lt;\u200954\u00a0years had a threefold increased risk of death at 1-year amongst HLH associated with malignancy compared to auto-immune. However, predicted 1-year survival decreased markedly with age in those with auto-immune (age 0\u201314, 84%; 15\u201354, 73%; &gt;\u200955, 27%) such that among those\u2009&gt;\u200955\u00a0years, survival was as poor as for patients with haematological malignancy. One-year survival following a diagnosis of HLH varies considerably by age, gender and associated comorbidity. Survival was better in those with auto-immune diseases among the young and middle age groups compared to those with an underlying malignancy, whereas in older age groups survival was uniformly poor regardless of the underlying disease process.<\/jats:p>","DOI":"10.1186\/s13045-023-01434-4","type":"journal-article","created":{"date-parts":[[2023,5,26]],"date-time":"2023-05-26T15:02:19Z","timestamp":1685113339000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["1-year survival in haemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003\u20132018"],"prefix":"10.1186","volume":"16","author":[{"given":"Joe","family":"West","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Stilwell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanhua","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu","family":"Ban","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Bythell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Card","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Lanyon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasanta","family":"Nanduri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Rankin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Bishton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin","family":"Crooks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,5,26]]},"reference":[{"issue":"9927","key":"1434_CR1","doi-asserted-by":"publisher","first-page":"1503","DOI":"10.1016\/S0140-6736(13)61048-X","volume":"383","author":"M Ramos-Casals","year":"2014","unstructured":"Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383(9927):1503\u201316.","journal-title":"Lancet"},{"issue":"4","key":"1434_CR2","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1111\/joim.13432","volume":"291","author":"J West","year":"2022","unstructured":"West J, Card TR, Bishton MJ, Lanyon P, Ban L, Bythell M, et al. Incidence and survival of haemophagocytic lymphohistiocytosis: a population-based cohort study from England. J Intern Med. 2022;291(4):493\u2013504.","journal-title":"J Intern Med"},{"key":"1434_CR3","doi-asserted-by":"publisher","first-page":"e797","DOI":"10.1097\/HS9.0000000000000797","volume":"6","author":"J West","year":"2022","unstructured":"West J, Stilwell P, Liu H, Ban L, Bythell M, Card TR, et al. Temporal trends in the incidence of haemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003\u20132018. HemaSphere. 2022;6:e797.","journal-title":"HemaSphere"},{"issue":"17","key":"1434_CR4","doi-asserted-by":"publisher","first-page":"3229","DOI":"10.1002\/cncr.30826","volume":"123","author":"N Daver","year":"2017","unstructured":"Daver N, McClain K, Allen CE, Parikh SA, Otrock Z, Rojas-Hernandez C, et al. A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults. Cancer. 2017;123(17):3229\u201340.","journal-title":"Cancer"},{"issue":"3","key":"1434_CR5","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1111\/bjh.13837","volume":"172","author":"AM Schram","year":"2016","unstructured":"Schram AM, Comstock P, Campo M, Gorovets D, Mullally A, Bodio K, et al. Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years. Br J Haematol. 2016;172(3):412\u20139.","journal-title":"Br J Haematol"},{"issue":"17","key":"1434_CR6","doi-asserted-by":"publisher","first-page":"4577","DOI":"10.1182\/blood-2011-06-356261","volume":"118","author":"H Trottestam","year":"2011","unstructured":"Trottestam H, Horne A, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011;118(17):4577\u201384.","journal-title":"Blood"},{"issue":"18","key":"1434_CR7","doi-asserted-by":"publisher","first-page":"2857","DOI":"10.1002\/cncr.30084","volume":"122","author":"GN Tamamyan","year":"2016","unstructured":"Tamamyan GN, Kantarjian HM, Ning J, Jain P, Sasaki K, McClain KL, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in adults: relation to hemophagocytosis, characteristics, and outcomes. Cancer. 2016;122(18):2857\u201366.","journal-title":"Cancer"},{"issue":"4","key":"1434_CR8","doi-asserted-by":"publisher","first-page":"1093","DOI":"10.1093\/ije\/dyx015","volume":"46","author":"A Herbert","year":"2017","unstructured":"Herbert A, Wijlaars L, Zylbersztejn A, Cromwell D, Hardelid P. Data resource profile: hospital episode statistics admitted patient care (HES APC). Int J Epidemiol. 2017;46(4):1093\u2013i.","journal-title":"Int J Epidemiol"},{"issue":"1","key":"1434_CR9","doi-asserted-by":"publisher","first-page":"16-h","DOI":"10.1093\/ije\/dyz076","volume":"49","author":"KE Henson","year":"2020","unstructured":"Henson KE, Elliss-Brookes L, Coupland VH, Payne E, Vernon S, Rous B, et al. Data resource profile: national cancer registration dataset in England. Int J Epidemiol. 2020;49(1):16-h.","journal-title":"Int J Epidemiol"},{"issue":"6","key":"1434_CR10","doi-asserted-by":"publisher","first-page":"1039","DOI":"10.1111\/bjh.17768","volume":"194","author":"MJ Bishton","year":"2021","unstructured":"Bishton MJ, Stilwell P, Card TR, Lanyon P, Ban L, Elliss-Brookes L, et al. A validation study of the identification of haemophagocytic lymphohistiocytosis in England using population-based health data. Br J Haematol. 2021;194(6):1039\u201344.","journal-title":"Br J Haematol"},{"key":"1434_CR11","volume-title":"R: a language and environment for statistical computing","author":"Team RC","year":"2021","unstructured":"Team RC. R: a language and environment for statistical computing. Vienna: R Foundation for Statistical Computing; 2021."}],"container-title":["Journal of Hematology &amp; Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13045-023-01434-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13045-023-01434-4\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13045-023-01434-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,26]],"date-time":"2023-05-26T15:07:05Z","timestamp":1685113625000},"score":1,"resource":{"primary":{"URL":"https:\/\/jhoonline.biomedcentral.com\/articles\/10.1186\/s13045-023-01434-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,26]]},"references-count":11,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,12]]}},"alternative-id":["1434"],"URL":"https:\/\/doi.org\/10.1186\/s13045-023-01434-4","relation":{},"ISSN":["1756-8722"],"issn-type":[{"value":"1756-8722","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,26]]},"assertion":[{"value":"10 March 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 March 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 May 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"No consent was obtained from individuals for this study as the data were collected and analysed under the National Disease Registries Directions 2021, made in accordance with sections 254(1) and 254(6) of the 2012 Health and Social Care Act. Ethical approval for this study was therefore not required per the definition of research according to the UK Policy Framework for Health and Social Care Research. The protocol was approved by the joint NDRS project board (reference PPF1920_027).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"All authors had financial support from Histio UK for the submitted work in the form of a research grant to the University of Nottingham; Dr. Lanyon is recipient of a research grant for an unrelated study from Vifor Pharma. Vifor Pharma had no influence on the design, conduct, or interpretation of this study. All authors declare no other authors declare no other relationships or activities that could appear to have influenced the submitted work.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"56"}}