{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T03:53:20Z","timestamp":1789012400003,"version":"build-2803163510"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,11,17]],"date-time":"2011-11-17T00:00:00Z","timestamp":1321488000000},"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":["Acta Paediatrica"],"published-print":{"date-parts":[[2012,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Aim:\u2002<\/jats:bold> Haemophagocytic lymphohistiocytosis (HLH) is a life\u2010threatening disturbance of immunoregulation. HLH comprises primary and acquired forms with different disease severity. A large proportion of deaths occur early into treatment. We investigated association with early death for laboratory and clinical parameters before the start of and 2 weeks into therapy.<\/jats:p><jats:p><jats:bold>Methods:\u2002<\/jats:bold> A total of 232 children from Scandinavia, Germany or Italy, fulfilling diagnostic criteria and\/or with familial disease and\/or HLH\u2010causing mutations, receiving HLH treatment 1994\u20132008 were included. The relation between clinical findings and early pre\u2010transplant death was examined using the Cox proportional hazards model, with a 4\u2010month right\u2010truncation of the outcome. Patients were censored at last follow\u2010up or transplant. Statistically significant predictors were adjusted for sex, age and each other.<\/jats:p><jats:p><jats:bold>Results:\u2002<\/jats:bold> The following features were significantly associated with adverse outcome: hyperbilirubinaemia (&gt;50\u2003\u03bcmol\/L; adjusted hazard ratio (aHR) 3.2; 95% confidence interval 1.3\u20138.1, p\u2003=\u20030.011), hyperferritinaemia (&gt;2000\u2003\u03bcg\/L; aHR 3.2; 1.2\u20138.6, p\u2003=\u20030.019), cerebrospinal fluid pleocytosis (&gt;100\u2003\u00d7\u200310<jats:sup>6<\/jats:sup>\/L; aHR 5.1; 1.4\u201318.5, p\u2003=\u20030.012) at diagnosis, and thrombocytopenia (&lt;40\u2003\u00d7\u200310<jats:sup>9<\/jats:sup>\/L; aHR 3.4; 1.1\u201310.7, p\u2003=\u20030.033), and hyperferritinaemia (&gt;2000\u2003\u03bcg\/L; aHR 10.6; 1.2\u201396.4, p\u2003=\u20030.037) 2\u2003weeks into therapy. Non\u2010improvement of fever, anaemia and\/or thrombocytopenia also had adverse impact.<\/jats:p><jats:p><jats:bold>Conclusion:\u2002<\/jats:bold> There seem to be easily available clinical predictors of early mortality in HLH patients, which may help guide treatment decisions.<\/jats:p>","DOI":"10.1111\/j.1651-2227.2011.02501.x","type":"journal-article","created":{"date-parts":[[2011,10,24]],"date-time":"2011-10-24T08:37:29Z","timestamp":1319445449000},"page":"313-318","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":74,"title":["Risk factors for early death in children with haemophagocytic 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