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. 2023 Apr 25;13(1):62.
doi: 10.1038/s41408-023-00831-9.

Transformation and survival in marginal zone lymphoma: a Finnish nationwide population-based study

Ilja Kalashnikov  1   2   3 , Tomas Tanskanen  4 , Leevi Viisanen  1   2   3 , Nea Malila  4 , Sirkku Jyrkkiö  5 , Sirpa Leppä  6   7   8
Affiliations

Transformation and survival in marginal zone lymphoma: a Finnish nationwide population-based study

Ilja Kalashnikov et al. Blood Cancer J. .

Abstract

Marginal zone lymphoma (MZL) is an indolent B-cell malignancy with heterogeneous anatomical and clinical presentation. While MZLs are generally associated with long survival, some patients experience histological transformation to aggressive large B-cell lymphoma. Population-based long-term data on the transformation of MZL is limited. We conducted a nationwide population-based study to estimate the risk of transformation and relative survival in patients diagnosed with MZL in Finland from 1995-2018. We identified a total of 1454 patients with MZL from the Finnish Cancer Registry (FCR). The cumulative incidence of transformation was 4.7% (95% CI, 3.6-6.2) at 10 years. The highest incidence of transformation was observed in the patients with splenic MZL (14.0%; 95% CI, 8.6-22.7). The transformation was associated with a substantially increased risk of death (HR, 5.18; 95% CI, 3.58-7.50). Ten-year relative survival was 79% (95% CI, 73‒83%). Transformation, nodal MZL subtype, and older age at diagnosis were associated with increased excess mortality, whereas patients diagnosed at a later calendar period had a lower excess risk of death. We conclude that transformation resulted in a substantially increased mortality irrespective of MZL subtype compared with the patients without transformation. Our results also suggest a reduction in excess mortality in recent years.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1. Cumulative incidence of transformation in patients diagnosed with marginal zone lymphoma in Finland in 1995–2018.
A Cumulative incidence of transformation for the whole cohort with 95% confidence intervals; B Cumulative incidence of transformation stratified by marginal zone lymphoma subtype. Estimates for unclassifiable subtypes are not shown. MZL marginal zone lymphoma, EMZL extranodal marginal zone lymphoma.
Fig. 2
Fig. 2. Stacked cumulative incidence curves for patients diagnosed with marginal zone lymphoma (MZL) at age 65 years in Finland in 2008.
A Female, gastric extranodal marginal zone lymphoma (EMZL); B female, non-gastric EMZL; C female, splenic MZL; D female, nodal MZL; E male, gastric EMZL; F male, non-gastric EMZL; G male, splenic MZL; H male, nodal MZL.
Fig. 3
Fig. 3. Hazard ratio for death (on a logarithmic scale on the y-axis) by the time from transformation.
The dashed lines indicate 95% confidence intervals.
Fig. 4
Fig. 4. Relative survival in patients diagnosed with marginal zone lymphoma in Finland in 1995–2018.
A Ten-year age-standardized relative survival for 1995–2018; B Ten-year age-standardized relative survival for 1995–2006 and 2007–2018; C Ten-year age-specific relative survival; D Ten-year age-standardized relative survival by subtype. MZL marginal zone lymphoma, EMZL extranodal marginal zone lymphoma.
See this image and copyright information in PMC

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