Evidence map›Paper›PMID 42668817›Full record

ReviewCureus2026

Prognostic Factors Associated With Mortality in Adult Hemophagocytic Lymphohistiocytosis: A Systematic Review and Meta-Analysis.

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

Abstract readReview
In one paragraph

Review in Cureus, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Shaza Bakri Osman ElsayedRheumatology, Croydon Health Services NHS Trust, London, GBR.
Hoyam AtitallaRheumatology, Basingstoke and North Hampshire Hospital, Hampshire Hospitals NHS Foundation Trust, Basingstoke, GBR.
Salma Elrayah Yousif AhmedInternal Medicine, MedicFirst, Melbourne, AUS.
Mohamed Kamal Gomaa AbdelfattahRheumatology, King Salman Specialist Hospital, Hail, SAU.
Mahmoud Abdelghany Eldyasty Ahmed EidDermatology, Al-Azhar University, Cairo, EGY.
Lobaba Mubarak Saidahmed AhmedInternal Medicine, Shendi University, Riyadh, SAU.
Eltayeb Osman Elfaki OmerEndocrinology, Dr. Sulaiman Al Habib Hospital, Riyadh, SAU.
Mohamed Sobhy Mahmoud RezkRheumatology, Faculty of Medicine, Al-Azhar University, Cairo, EGY.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

ferritinhemophagocytic lymphohistiocytosismalignancymeta-analysismortalityprognosissystematic review

Identifiers

PMID42668817
PMCPMC13525352

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.