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. 2023 Sep 1;13(1):130.
doi: 10.1038/s41408-023-00903-w.

A population-based study of transformed marginal zone lymphoma: identifying outcome-related characteristics

Johanna A A Bult  1 , Francien Huisman  1 , Yujie Zhong  1   2 , Nick Veltmaat  1 , Joost Kluiver  2 , Sanne H Tonino  3 , Joost S P Vermaat  4 , Martine E D Chamuleau  3 , Arjan Diepstra  2 , Anke van den Berg  2 , Wouter J Plattel  1 , Mirian Brink  5 , Marcel Nijland  6
Affiliations

A population-based study of transformed marginal zone lymphoma: identifying outcome-related characteristics

Johanna A A Bult et al. Blood Cancer J. .

Abstract

Histological transformation of marginal zone lymphoma (tMZL) into diffuse large B-cell lymphoma is associated with poor outcomes. Clinical characteristics associated with transformation risk and outcome after transformation are largely unknown due to scarcity of data. In this population-based study, competing risk analyses were performed to elucidate clinical characteristics associated with developing transformation among 1793 MZL patients using the Netherlands Cancer Registry. Cox regression analyses were performed to elucidate clinical characteristics associated with risk of relapse and mortality after transformation. Transformation occurred in 75 (4%) out of 1793 MZL patients. Elevated LDH and nodal MZL subtype at MZL diagnosis were associated with an increased risk, and radiotherapy with a reduced risk of developing tMZL. Most tMZL patients received R-(mini)CHOP (n = 53, 71%). Age >60 years and (immuno)chemotherapy before transformation were associated with an increased risk of relapse and mortality after transformation. Two-year progression-free survival (PFS) and overall survival (OS) were 66% (95% CI 52-77%) and 75% (95% CI 62-85%) for R-(mini)CHOP-treated tMZL patients, as compared to a PFS and OS both of 41% (95% CI 19-63%) for patients treated otherwise. Our study offers comprehensive insights into characteristics associated with transformation and survival after transformation, thereby optimizing guidelines and patient counseling.

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Conflict of interest statement

ST received consultation fees from van Takeda, Incyte Biosciences, and Roche Pharma. MC received financial support for clinical trials from Celgene, Bristol-Myers Squibb, and Gilead. AD received research funding from Takeda. The remaining authors declare no competing financial interests.

Figures

Fig. 1
Fig. 1. Cumulative incidence of transformation in 1 793 marginal zone lymphoma (MZL) patients between 2014 and 2020.
A Cumulative incidence of transformation for the entire cohort of MZL patients; B Cumulative incidence of transformation per MZL subtype, NMZL nodal MZL, SMZL splenic MZL, EMZL extranodal MZL.
Fig. 2
Fig. 2. Overall survival (OS-1) of marginal zone lymphoma patients with or without a transformation.
The black line indicates transformed MZL patients and the blue line indicates non-transformed MZL patients.
Fig. 3
Fig. 3. Progression-free survival and overall survival of transformed marginal zone lymphoma patients.
The black line indicates the overall-survival after tMZL diagnosis, and the blue line indicates the progression-free survival after tMZL diagnosis.
Fig. 4
Fig. 4. Forest plot of multivariable analysis for risk of relapse and mortality among transformed marginal zone lymphoma patients.
Hazard ratios and 95% confidence intervals (CIs) are presented in blue for risk factors associated with relapse and in black for risk factors associated with mortality.
See this image and copyright information in PMC

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