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Published in: Journal of Hematology & Oncology 1/2023

Open Access 01-12-2023 | Chronic Inflammatory Bowel Disease | Correspondence

1-year survival in haemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003–2018

Authors: Joe West, Peter Stilwell, Hanhua Liu, Lu Ban, Mary Bythell, Tim Card, Peter Lanyon, Vasanta Nanduri, Judith Rankin, Mark Bishton, Colin Crooks

Published in: Journal of Hematology & Oncology | Issue 1/2023

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Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a lethal syndrome of excessive immune activation. We undertook a nationwide study in England of all cases of HLH diagnosed between 2003 and 2018, using linked electronic health data from hospital admissions and death certification. We modelled interactions between demographics and comorbidities and estimated one-year survival by calendar year, age group, gender and comorbidity (haematological malignancy, auto-immune, other malignancy) using Cox regression. There were 1628 people with HLH identified. Overall, crude one-year survival was 50% (95% Confidence interval 48–53%) which varied substantially with age (0–4: 61%; 5–14: 76%; 15–54: 61%; > 55: 24% p < 0.01), sex (males, 46%, worse than females, 55% p < 0.01) and associated comorbidity (auto-immune, 69%, haematological malignancy 28%, any other malignancy, 37% p < 0.01). Those aged < 54 years had a threefold increased risk of death at 1-year amongst HLH associated with malignancy compared to auto-immune. However, predicted 1-year survival decreased markedly with age in those with auto-immune (age 0–14, 84%; 15–54, 73%; > 55, 27%) such that among those > 55 years, survival was as poor as for patients with haematological malignancy. One-year survival following a diagnosis of HLH varies considerably by age, gender and associated comorbidity. Survival was better in those with auto-immune diseases among the young and middle age groups compared to those with an underlying malignancy, whereas in older age groups survival was uniformly poor regardless of the underlying disease process.
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Metadata
Title
1-year survival in haemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003–2018
Authors
Joe West
Peter Stilwell
Hanhua Liu
Lu Ban
Mary Bythell
Tim Card
Peter Lanyon
Vasanta Nanduri
Judith Rankin
Mark Bishton
Colin Crooks
Publication date
01-12-2023
Publisher
BioMed Central
Published in
Journal of Hematology & Oncology / Issue 1/2023
Electronic ISSN: 1756-8722
DOI
https://doi.org/10.1186/s13045-023-01434-4

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