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

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

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.

Citation

West, J., Stilwell, P., Liu, H., Ban, L., Bythell, M., Card, T., …Crooks, C. (2023). 1-year survival in haemophagocytic lymphohistiocytosis: a nationwide cohort study from England 2003–2018. Journal of Hematology and Oncology, 16, Article 56. https://doi.org/10.1186/s13045-023-01434-4

• Journal Article Type: Article
• Acceptance Date: Mar 29, 2023
• Online Publication Date: May 26, 2023
• Publication Date: May 26, 2023
• Deposit Date: Apr 3, 2023
• Publicly Available Date: May 26, 2023
• Journal: Journal of Hematology and Oncology
• Electronic ISSN: 1756-8722
• Publisher: Springer Verlag
• Peer Reviewed: Peer Reviewed
• Volume: 16
• Article Number: 56
• DOI: https://doi.org/10.1186/s13045-023-01434-4
• Keywords: HLH, Survival, Blood cancer
• Public URL: https://nottingham-repository.worktribe.com/output/19208651
• Publisher URL: https://jhoonline.biomedcentral.com/articles/10.1186/s13045-023-01434-4
• Additional Information: Received: 10 March 2023; Accepted: 29 March 2023; First Online: 26 May 2023; : ; : No consent was obtained from individuals for this study as the data were collected and analysed under the National Disease Registries Directions 2021, made in accordance with sections 254(1) and 254(6) of the 2012 Health and Social Care Act. Ethical approval for this study was therefore not required per the definition of research according to the UK Policy Framework for Health and Social Care Research. The protocol was approved by the joint NDRS project board (reference PPF1920_027).; : Not applicable.; : All authors had financial support from Histio UK for the submitted work in the form of a research grant to the University of Nottingham; Dr. Lanyon is recipient of a research grant for an unrelated study from Vifor Pharma. Vifor Pharma had no influence on the design, conduct, or interpretation of this study. All authors declare no other authors declare no other relationships or activities that could appear to have influenced the submitted work.