Trends in mortality due to haemophagocytic lymphohistiocytosis across 29 European countries from 2011 to 2021: a retrospective, international, population-based study
Authors' conclusion
Recorded rates of mortality due to haemophagocytic lymphohistiocytosis have nearly doubled over the past decade in Europe.
Study context
- Population
- Deaths with HLH (ICD-10 D76.1 or D76.2) as underlying cause in 29 European countries, 2011–2021deaths, 29 countries
- Participants
- 3,345 deaths
- Outcome
- Mortality due to HLH (death registration rates)
- Follow-up
- 2011–2021
Methodological detailsdeath certificate data (EUROSTAT), Poisson regression, direct standardisation, 5 of 34 countries excluded because data censored due to small numbers of deaths
- Death certificate data (EUROSTAT)
- Poisson regression
- Direct standardisation
- 5 of 34 countries excluded because data censored due to small numbers of deaths
Main finding
As reported in the abstract
HLH mortality per 10 000 000 person-years
- Crude mortality, 2011
- 3.9 per 10 000 000 person-years
- Crude mortality, 2021
- 6.6 per 10 000 000 person-years
- Age-sex-standardised mortality rate across Europe
- 4.7 (95% CI 3.0-6.4) per 10 000 000 person-years
- Highest rate: France
- 10.1, 0.0-27.3
- Lowest rate: Romania
- 0.5, 0.0-13.6
Observational data: associations, not effects.
Further findings
| Outcome | Adjusted rate ratio (95% CI) | |
|---|---|---|
| Mortality, malevs female | 1.5 (1.4–1.6) | |
| 0.512Adjusted rate ratio (log scale) |
Crude mortality by age
- Infants aged 0-4 years
- 17.5, 95% CI 16.1-19.0
- Adults aged 80-85 years
- 15.6, 13.7-17.6
Correlation between HLH mortality and research activity (publications)
- Pearson's correlation coefficient
- 0.4968; p=0.012
Original publication
Trends in mortality due to haemophagocytic lymphohistiocytosis across 29 European countries from 2011 to 2021: a retrospective, international, population-based study. Lancet Rheumatol. Published 29 November 2025. doi:10.
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