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

• Published in: Journal of hematology and oncology Vol. 16; no. 1; p. 56
• Main Authors: West, Joe, Stilwell, Peter, Liu, Hanhua, Ban, Lu, Bythell, Mary, Card, Tim, Lanyon, Peter, Nanduri, Vasanta, Rankin, Judith, Bishton, Mark, Crooks, Colin
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 26-05-2023; BioMed Central; BMC
• Subjects: Aged; Autoimmune diseases; Bans; Blood cancer; Blood cancers; Bowel disease; Cancer; Cohort analysis; Cohort Studies; Comorbidity; Correspondence; Cytokines; Datasets; Female; Gender; Hematologic Neoplasms - complications; Hematologic Neoplasms - epidemiology; Hematology; HLH; Hospitals; Humans; Immune response; Immunological diseases; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - epidemiology; Male; Malignancy; Middle Aged; Mortality; Neoplasms - complications; Oncology; Population; Registration; Retrospective Studies; Survival; Viral infections; United Kingdom; United Kingdom > UK; England; HLH; Survival; Blood cancers
• ISSN: 1756-8722; 1756-8722
• DOI: 10.1186/s13045-023-01434-4

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.