Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study

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 limi...

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Main Authors: Ilja Kalashnikov, Tomas Tanskanen, Janne Pitkäniemi, Nea Malila, Sirkku Jyrkkiö, Sirpa Leppä
Format: Article
Language:English
Published: Nature Publishing Group 2021-12-01
Series:Blood Cancer Journal
Online Access:https://doi.org/10.1038/s41408-021-00586-1
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author Ilja Kalashnikov
Tomas Tanskanen
Janne Pitkäniemi
Nea Malila
Sirkku Jyrkkiö
Sirpa Leppä
author_facet Ilja Kalashnikov
Tomas Tanskanen
Janne Pitkäniemi
Nea Malila
Sirkku Jyrkkiö
Sirpa Leppä
author_sort Ilja Kalashnikov
collection DOAJ
description 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.
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spelling doaj.art-7cdbd33b83c44b75b830291c76a2b6ec2022-12-21T20:20:21ZengNature Publishing GroupBlood Cancer Journal2044-53852021-12-0111121610.1038/s41408-021-00586-1Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based studyIlja Kalashnikov0Tomas Tanskanen1Janne Pitkäniemi2Nea Malila3Sirkku Jyrkkiö4Sirpa Leppä5Research Program Unit, Applied Tumor Genomics, Faculty of Medicine, University of HelsinkiFinnish Cancer Registry, Institute for Statistical and Epidemiological Cancer ResearchFinnish Cancer Registry, Institute for Statistical and Epidemiological Cancer ResearchFinnish Cancer Registry, Institute for Statistical and Epidemiological Cancer ResearchDepartment of Oncology and Radiotherapy, Turku University HospitalResearch Program Unit, Applied Tumor Genomics, Faculty of Medicine, University of HelsinkiAbstract 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.https://doi.org/10.1038/s41408-021-00586-1
spellingShingle Ilja Kalashnikov
Tomas Tanskanen
Janne Pitkäniemi
Nea Malila
Sirkku Jyrkkiö
Sirpa Leppä
Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
Blood Cancer Journal
title Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
title_full Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
title_fullStr Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
title_full_unstemmed Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
title_short Transformation and outcome of nodular lymphocyte predominant Hodgkin lymphoma: a Finnish Nationwide population-based study
title_sort transformation and outcome of nodular lymphocyte predominant hodgkin lymphoma a finnish nationwide population based study
url https://doi.org/10.1038/s41408-021-00586-1
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