Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare B-cell malignancy associated with excellent survival. However, some patients experience histological transformation into aggressive large B-cell lymphoma. Population-based data on transformation in patients with NLPHL is limited. We conducted a nationwide population-based study to estimate the risk of transformation and relative survival in patients diagnosed with NLPHL in Finland between 1995 and 2018. We identified a total of 453 patients (median age, 48 years; 76% males) with the incident NLPHL from the Finnish Cancer Registry. The cumulative incidence of transformation was 6.3% (95% CI, 4.2-9.6) at 10 years. After adjusting for sex, age and year of diagnosis, transformation was associated with a substantially increased risk of death (HR 8.55, 95% CI 4.49−16.3). Ten-year relative survival was 94% (95% CI, 89%‒100%). The patients diagnosed at a later calendar year had lower excess risk of death (HR, 0.38 per 10-year increase; 95% CI, 0.15‒0.98). We conclude that while the 10-year relative survival for the patients with NLPHL was excellent in this large population-based cohort for the entire study period, transformation resulted in a substantially increased mortality compared with the patients without transformation. Our results also suggest a reduction in excess mortality over time.

结节性淋巴细胞为主的霍奇金淋巴瘤 (NLPHL) 是一种罕见的 B 细胞恶性肿瘤，具有良好的生存率。然而，一些患者经历组织学转化为侵袭性大 B 细胞淋巴瘤。关于 NLPHL 患者转化的基于人群的数据有限。我们进行了一项全国性的基于人群的研究，以估计 1995 年至 2018 年间在芬兰被诊断为 NLPHL 的患者的转化风险和相对生存率。我们确定了共有 453 名患者（中位年龄，48 岁；76% 为男性）发生了该事件来自芬兰癌症登记处的 NLPHL。10 年时转化的累积发生率为 6.3% (95% CI, 4.2-9.6)。在对性别、年龄和诊断年份进行调整后，转化与死亡风险显着增加相关（HR 8.55，95% CI 4.49-16.3）。十年相对生存率为 94%（95% CI，89%-100%）。在较晚日历年诊断的患者的超额死亡风险较低（HR，每 10 年增加 0.38；95% CI，0.15-0.98）。我们得出的结论是，虽然 NLPHL 患者的 10 年相对生存率在整个研究期间在这个基于人群的大型队列中非常出色，但与未转化的患者相比，转化导致死亡率显着增加。我们的结果还表明，随着时间的推移，超额死亡率会降低。与未转化的患者相比，转化导致死亡率显着增加。我们的结果还表明，随着时间的推移，超额死亡率会降低。与未转化的患者相比，转化导致死亡率显着增加。我们的结果还表明，随着时间的推移，超额死亡率会降低。