Authors
Shaza Bakri Osman Elsayed, Hoyam Atitalla, Salma Elrayah Yousif Ahmed, Mohamed Kamal Gomaa Abdelfattah, Mahmoud Abdelghany Eldyasty Ahmed Eid, Lobaba Mubarak Saidahmed Ahmed, Eltayeb Osman Elfaki Omer, Mohamed Sobhy Mahmoud Rezk
Published in
Cureus. Volume 18. Issue 7. Pages e113630. Epub Jul 29, 2026.
Abstract
Adult hemophagocytic lymphohistiocytosis (HLH) carries a high case-fatality rate, yet reported prognostic factors are discordant and have never been pooled. The aim of this study was to perform the first systematic review and meta-analysis of prognostic factors for mortality in adult HLH. PubMed/MEDLINE, Embase, Web of Science, and the Cochrane Library were searched (from database inception to February 15, 2026) for observational cohort studies of adults (≥18 years) with HLH diagnosed by the HLH-2004 criteria and/or the HScore, reporting mortality against at least one candidate prognostic factor. Risk of bias was appraised using the Quality In Prognosis Studies (QUIPS) tool, and certainty using GRADE. Random-effects meta-analyses (DerSimonian-Laird with Hartung-Knapp correction) were pre-specified for factors reported in at least three cohorts. Reporting followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020. A total of 18 cohorts (3,570 adults) were included. Pooled mortality was 49.5% (95% CI: 41.1-57.8), rising from 34.4% (29.0-40.0) within 30 days to 57.3% (47.9-66.4) at longest follow-up (p<0.001). Malignancy-associated HLH carried higher mortality than other triggers (47.1% vs. 32.2%; 12 cohorts, n=2,373; pooled odds ratio 2.53, 95% CI: 1.55-4.12; I²=66.7%). Older age (2.23, 95% CI: 1.57-3.15; I²=0%), hyperferritinemia (3.07, 95% CI: 1.07-8.82), and elevated lactate dehydrogenase (2.02, 95% CI: 1.05-3.88) independently predicted death; thrombocytopenia and prolonged activated partial thromboplastin time were directionally harmful but imprecise. Egger's test indicated small-study effects (p=0.014). Certainty was low to moderate. Approximately half of adults with HLH die following diagnosis, with nearly one-third of deaths occurring within 30 days. A malignant trigger approximately doubles the odds of death; age, ferritin, and lactate dehydrogenase add independent information. Prospective, multinational registry data with pre-specified thresholds are needed.
PMID:
42668817
Bibliographic data and abstract were imported from PubMed on 30 Aug 2026.
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