Population and Lifespan Sciences, University of Nottingham, Nottingham, UK.
NIHR Biomedical Research Centre, Nottingham University Hospitals NHS Trust and University of Nottingham, Nottingham, UK.
J Intern Med. 2022 Apr;291(4):493-504. doi: 10.1111/joim.13432. Epub 2021 Dec 22.
Haemophagocytic lymphohistiocytosis (HLH) is a rare hyper-inflammatory condition with poor outcomes.
Few population-based estimates of the incidence and survival in adults exist. We aimed to provide these data for England.
We used population-based linked data from primary care, secondary care, cancer registries and mortality databases in England to identify people diagnosed with HLH between 1 January 2000 and 31 December 2016. We calculated annual incidence rates by age and sex, modelled change in incidence over time with Poisson regression, calculated overall 1-year survival using Kaplan-Meier methods and estimated adjusted hazard ratios (HRs) of death using a Cox proportional hazards model.
We identified 214 patients with HLH. The reported age and sex-adjusted incidence increased twofold over the period, from around one to around two per million. Incidence was highest in those below 1 year (14.6 per million) and ≥75 years (2.2 per million), and lowest in those aged 15-44 years (0.8 per million). One-year survival varied by age and sex from 77% (95% confidence interval [CI] 63%-86%) in those <15 years to 30% (95% CI 14%-49%) in those ≥75. In patients with haematological cancer, the adjusted HR for death was 2.60 (95% CI 1.45-4.66) compared to patients with no malignant or rheumatological disease.
The incidence of HLH diagnosis in England has increased between 2000 and 2016 and occurs in all ages with varying underlying diseases. One-year survival varies substantially, being particularly poor in those aged over 75 years and those with haematological malignancy.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的炎症过度活跃的病症，预后较差。
目前针对成人 HLH 的发病率和存活率的人群研究较少。本研究旨在提供英格兰的相关数据。
我们使用英格兰初级保健、二级保健、癌症登记处和死亡率数据库的人群关联数据，识别了 2000 年 1 月 1 日至 2016 年 12 月 31 日期间诊断为 HLH 的人群。我们按年龄和性别计算了每年的发病率，使用泊松回归模型分析发病率随时间的变化，使用 Kaplan-Meier 方法计算了总的 1 年生存率，并使用 Cox 比例风险模型估计了死亡的调整后风险比（HR）。
我们共确定了 214 例 HLH 患者。在此期间，年龄和性别校正后的发病率增加了一倍，从大约每百万人口 1 例增加到大约每百万人口 2 例。发病率在 1 岁以下（14.6/百万）和≥75 岁（2.2/百万）人群中最高，在 15-44 岁人群中（0.8/百万）最低。1 年生存率因年龄和性别而异，15 岁以下患者为 77%（95%置信区间 [CI] 63%-86%），75 岁以上患者为 30%（95% CI 14%-49%）。与无恶性或风湿性疾病的患者相比，血液系统癌症患者的死亡调整 HR 为 2.60（95% CI 1.45-4.66）。
2000 年至 2016 年期间，英格兰 HLH 的诊断发病率有所增加，且可发生于各个年龄段，存在不同的潜在疾病。1 年生存率差异很大，75 岁以上患者和血液系统恶性肿瘤患者的生存率尤其差。
