Affiliation:
1. University of Nottingham Nottingham UK
2. Nottingham University Hospitals NHS Trust Nottingham UK
3. National Disease Registration Service NHS England Leeds UK
4. NIHR Nottingham Biomedical Research Centre Nottingham University Hospitals NHS Trust and the University of Nottingham Nottingham UK
5. Department of Clinical Medicine Aarhus University Aarhus Denmark
Abstract
SummaryTo address the lack of contemporary population‐based epidemiological studies of hepatosplenic T‐cell lymphoma (HSTCL), we undertook a population‐based study of ICD‐O‐3‐coded HSTCL in England. We used the National Cancer Registration Dataset and linked datasets on hospital admissions, Systemic Anti‐Cancer Therapy, socio‐demographics, comorbidities and death, identifying cases from 1 January 2013 to 31 December 2019 with survival data up to 5 January 2021. Crude and directly age‐standardised incidence rates per million persons per year were calculated. Crude and adjusted incidence rate ratios compared incidence between groups using Poisson regression. A Cox proportional hazards model estimated mortality risks adjusted for age, sex, ethnicity, deprivation and allogenic stem cell transplant (allo‐SCT; time varying). We identified 44 patients, mean age 42 years. Median survival was 11 months, and 1 and 5 year survivals were 48% (95% CI 29%–43%) and 22% (95% CI 12%–42%) respectively. The age‐standardised incidence was 0.1 per million/year. Incidence was higher in areas with greater deprivation (0.15 per million/year), and more cases than expected were in non‐White patients (39%). Non‐Whites had a twofold increased risk of death (adjusted hazard ratio 2.21 [95% CI 1.03–4.78]) even after adjusting for deprivation, younger age and allo‐SCT. In conclusion, ethnicity and socio‐economic status affect both the incidence and survival of HSTCL.