Clinical Characteristics, Treatment and Evolution of Splenic and Nodal Marginal Zone Lymphomas—Retrospective and Multicentric Analysis of Portuguese Centers

Rodrigues, Catarina Dantas; Peixeiro, Rita Pinho; Viegas, Diana; Chorão, Pedro; Couto, Maria Eduarda; Gaspar, C.; Fernandes, João Paulo S.; Alves, Daniela; Ribeiro, Lídia Alves; M, Pedro de Vasconcelos; Tomé, Ana; Badior, Margarida; Coelho, Henrique; Príncipe, Fernando; Chacim, Sérgio; Silva, Maria Gomes da; Coutinho, Rita

doi:10.1016/j.clml.2021.06.013

Cited by 4 publications

(3 citation statements)

References 25 publications

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“…The cumulative incidence of transformation in the current study is lower than previous studies with 5-year cumulative incidence rates of 2.5% to 15% and 10-year cumulative incidence rates of 4.7% to 18%. 13,14,[16][17][18][19][20][21][22] The difference in the transformation rates between our findings and those of these reports lacks a conclusive cause.…”

Section: Discussioncontrasting

confidence: 99%

Transformation to diffuse large B‐cell lymphoma and its impact on survival in patients with marginal zone lymphoma: A population‐based study

Du,

Wang,

et al. 2023

Intl Journal of Cancer

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Some patients with marginal zone lymphoma (MZL) experience histological transformation to diffuse large B‐cell lymphoma (DLBCL). Because of the paucity of long‐term data on transformation, we conducted a population‐based study to estimate the risk of transformation and its impact on survival in MZL. Using the Surveillance, Epidemiology and End Results database, we identified 23 221 patients with histology‐proven MZL between 2000 and 2018. Competing risk method, Kaplan‐Meier and Cox proportional hazards regression were performed to analyze time‐to‐event outcomes. Based on 420 events of transformation, the 10‐year cumulative incidence rate of transformation is 2.23% (95% CI: 2.00%‐2.46%) in MZL, 1.5% (95% CI: 1.3%‐1.8%), 2.7% (95% CI: 2.3%‐3.2%) and 5.8% (95% CI: 4.6%‐7.1%) in extranodal, nodal and splenic MZL (EMZL, NMZL and SMZL), respectively. Patients with SMZL (subdistribution hazard ratio [SHR], 2.96; 95% CI: 2.21‐3.96) or NMZL (SHR, 1.49; 95% CI: 1.17‐1.90) have a higher risk of transformation than those with EMZL. For each MZL subtype, patients with transformation had a significantly shorter overall survival. Patients with transformation >18 months since MZL diagnosis had longer OS than those who presented within 18 months (5‐year rate, 87.4% [95% CI: 83.7%‐91.2%] vs 47.9% [95% CI: 38.8%‐59.0%]; P < .001). Compared to patients with matched de novo DLBCL, those whose DLBCL was transformed from MZL had a shorter OS (5‐year rate, 56.6% [95% CI: 51.9%‐61.8%] vs 46.1% [95% CI: 40.9%‐51.9%]; P < .001). We concluded that patients with SMZL had the highest risk of transformation. Regardless of MZL subtype, transformation resulted in significantly increased mortality.

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Section: Discussioncontrasting

confidence: 99%

Transformation to diffuse large B‐cell lymphoma and its impact on survival in patients with marginal zone lymphoma: A population‐based study

Du,

Wang,

et al. 2023

Intl Journal of Cancer

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“…The low cumulative incidence of transformation as observed in the current study is in line with previous reports with 5-year cumulative incidence rates of 2.5–6.6% [ 4 , 5 , 7 , 8 , 10 ]. However, some studies have reported higher 5-year incidence rates, as much as 10% [ 6 , 9 ].…”

Section: Discussionsupporting

confidence: 93%

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

Bult,

Huisman,

Zhong

et al. 2023

Blood Cancer J.

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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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“…The patient in this report was diagnosed with WM and received BTK inhibitor monotherapy for two years. The treatment of WM has progressed from traditional treatment with benzodiazepine, CHOP, and the FC regimen to new treatments based on rituximab, proteasome inhibitors, and BTK inhibitors[ 3 ]. Over the past ten years, our centre has gradually increased the proportion of patients on new treatment regimens.…”

Section: Discussionmentioning

confidence: 99%

Secondary light chain amyloidosis with Waldenström’s macroglobulinemia and intermodal marginal zone lymphoma: A case report

Tang¹,

Fu²,

Lin³

2022

World J Clin Cases

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BACKGROUND The co-existence of Waldenström’s macroglobulinemia (WM) with internodal marginal zone lymphoma (INMZL) is rare and often associated with poor prognosis. CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment. A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years. Her INMZL was confirmed due to left cervical lymphadenopathy. The patient presented with oedema in both lower limbs one year ago, and was diagnosed with secondary light chain amyloidosis. Treatment with the BC regimen (rituximab 375 mg/m 2 monthly for 6-8 courses, and bendamustine 90 mg/m 2 per day × 2, monthly for six courses) was initiated, but not tolerated due to toxic side effects. Bortezomib-based therapy was given for two months, including bortezomib, dexamethasone, and zanubrutinb. Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission. CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.

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Clinical Characteristics, Treatment and Evolution of Splenic and Nodal Marginal Zone Lymphomas—Retrospective and Multicentric Analysis of Portuguese Centers

Cited by 4 publications

References 25 publications

Transformation to diffuse large B‐cell lymphoma and its impact on survival in patients with marginal zone lymphoma: A population‐based study

Transformation to diffuse large B‐cell lymphoma and its impact on survival in patients with marginal zone lymphoma: A population‐based study

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

Secondary light chain amyloidosis with Waldenström’s macroglobulinemia and intermodal marginal zone lymphoma: A case report

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