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

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 prima...

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Bibliographic Details
Published in:Journal of internal medicine 2022-04, Vol.291 (4), p.493-504
Main Authors: West, Joe, Card, Tim R., Bishton, Mark J., Lanyon, Peter, Ban, Lu, Bythell, Mary, Elliss‐Brookes, Lucy, Manson, Jessica J., Nanduri, Vasanta, Rankin, Judith, Tattersall, Rachel S., Crooks, Colin J.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Aged
Cancer
Cohort analysis
Cohort Studies
Confidence intervals
epidemiology
Hazard identification
Health care
Health hazards
Hematology
HLH
Humans
Incidence
Inflammation
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - epidemiology
Malignancy
Population studies
Population-based studies
Primary care
Proportional Hazards Models
Registries
Sex
Statistical analysis
Statistical models
Survival
Young Adult
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Summary: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
ISSN:0954-6820
1365-2796
DOI:10.1111/joim.13432