Incidence and survival of haemophagocytic lymphohistiocytosis: A population-based cohort study from England.

Autor: West J; Population and Lifespan Sciences, University of Nottingham, Nottingham, UK.; NIHR Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, Nottingham, UK.; National Disease Registration Service, NHS Digital, Leeds, UK., Card TR; Population and Lifespan Sciences, University of Nottingham, Nottingham, UK.; NIHR Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, Nottingham, UK., Bishton MJ; Department of Haematology, Nottingham City Hospital, Nottingham, UK., Lanyon P; Population and Lifespan Sciences, University of Nottingham, Nottingham, UK.; NIHR Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, Nottingham, UK., Ban L; Evidera by PPD, London, UK., Bythell M; National Disease Registration Service, NHS Digital, Leeds, UK., Elliss-Brookes L; National Disease Registration Service, NHS Digital, Leeds, UK., Manson JJ; Department of Rheumatology, University College London Hospitals, London, UK., Nanduri V; Department of Paediatrics, Watford General Hospital, Watford, UK., Rankin J; National Disease Registration Service, NHS Digital, Leeds, UK.; Population Health Sciences Institute, Newcastle University, Newcastle upon Tyne, UK., Tattersall RS; Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK., Crooks CJ; NIHR Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, Nottingham, UK.; National Disease Registration Service, NHS Digital, Leeds, UK.; Translational Medical Sciences, University of Nottingham, Nottingham, UK.
Jazyk: angličtina
Zdroj: Journal of internal medicine [J Intern Med] 2022 Apr; Vol. 291 (4), pp. 493-504. Date of Electronic Publication: 2021 Dec 22.
DOI: 10.1111/joim.13432
Abstrakt: Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare hyper-inflammatory condition with poor outcomes.
Objectives: Few population-based estimates of the incidence and survival in adults exist. We aimed to provide these data for England.
Methods: We used population-based linked data from primary care, secondary care, cancer registries and mortality databases in England to identify people diagnosed with HLH between 1 January 2000 and 31 December 2016. We calculated annual incidence rates by age and sex, modelled change in incidence over time with Poisson regression, calculated overall 1-year survival using Kaplan-Meier methods and estimated adjusted hazard ratios (HRs) of death using a Cox proportional hazards model.
Results: We identified 214 patients with HLH. The reported age and sex-adjusted incidence increased twofold over the period, from around one to around two per million. Incidence was highest in those below 1 year (14.6 per million) and ≥75 years (2.2 per million), and lowest in those aged 15-44 years (0.8 per million). One-year survival varied by age and sex from 77% (95% confidence interval [CI] 63%-86%) in those <15 years to 30% (95% CI 14%-49%) in those ≥75. In patients with haematological cancer, the adjusted HR for death was 2.60 (95% CI 1.45-4.66) compared to patients with no malignant or rheumatological disease.
Conclusion: The incidence of HLH diagnosis in England has increased between 2000 and 2016 and occurs in all ages with varying underlying diseases. One-year survival varies substantially, being particularly poor in those aged over 75 years and those with haematological malignancy.
(© 2021 The Association for the Publication of the Journal of Internal Medicine.)
Databáze: MEDLINE
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