Abstract
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.
Highlights
Worldwide, Hodgkin lymphoma (HL) accounted for an estimated 83 000 (0.4%) new cancer cases and 23 000 (0.2%) cancer deaths in 2020 [1]
We identified 453 patients diagnosed with Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) in Finland
Patients diagnosed with incident NLPHL (ICD-O-3 morphology code 9659/ 3) in Finland in 1995–2018 were retrieved from the Finnish Cancer Registry (FCR) database
Summary
Hodgkin lymphoma (HL) accounted for an estimated 83 000 (0.4%) new cancer cases and 23 000 (0.2%) cancer deaths in 2020 [1]. While the age-standardized incidence remained rather stable, the age-standardized mortality and disability adjusted lifeyear rates have steadily decreased since the 1990s [1, 2], resulting in excellent survival rates. Between 2014 and 2018 in the Nordic countries, 5-year relative survival was over 85%. In Finland, approximately 180 new HL cases are diagnosed annually [2, 3]. Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare subtype of HL with unique clinicopathological features, and an estimated incidence of 1-3 per million per year, representing 5-15% of all new HL cases [4,5,6,7]. There is a risk of late disease progression and histological transformation into large B-cell lymphoma [8, 9]
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