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. 2024 Oct;103(10):4211-4222.
doi: 10.1007/s00277-024-05688-x. Epub 2024 Mar 9.

Transformation risk and associated survival outcome of marginal zone lymphoma: A nationwide study

Xu Sun #  1 , He Li #  1 , Yuqi Yang  2 , Yijun Wu  3 , Kai Kang  3 , Qinyu Liu  1 , Ren Luo  3 , Li Wang  1 , Ailin Zhao  4 , Ting Niu  5
Affiliations

Transformation risk and associated survival outcome of marginal zone lymphoma: A nationwide study

Xu Sun et al. Ann Hematol. 2024 Oct.

Abstract

Histological transformation into an aggressive B-cell lymphoma indicates a poor survival outcome for patients with indolent marginal zone lymphoma (MZL), which has been less studied. Large-scale data with long-term follow-up to investigate MZL transformation is limited. Here, by reporting a US-Nationwide cohort of 30,619 MZL patients diagnosed between 2000 and 2019, we found that transformation occurred in 2.08% (N = 624) of MZL cases, with the transformation incidence of 3.1 per 1,000 person-years. Advanced Ann Arbor stage, nodal MZL (NMZL) and splenic MZL (SMZL) were associated with an elevated risk of transformation. Certain subtype-specific characteristics, such as non-gastric extra-nodal MZL (vs. gastric, HR, 1.51, 95%CI 1.13-2.04; p = 0.006), and receiving splenectomy for SMZL (HR, 2.04, 95%CI 1.28-3.26; p = 0.003), also indicated a higher risk of transformation. Besides, transformation independently increased the overall mortality risk (HR, 1.38, 95%CI 1.24-1.53, p < 0.001), especially the higher lymphoma-caused mortality risk (HR, 3.21, 95%CI 2.81-3.67, p < 0.001). Transformation was also associated with a higher percentage of lymphoma-caused deaths. The post-transformation prognostic analyses demonstrated that female gender and age ≥ 65 years independently affected patients' mortalities. These findings, based on the largest cohort to date, contribute to a better understanding of transformed MZL, and provide valuable reference points for guidelines and patient counseling.

Keywords: Marginal zone lymphoma; Population-based study; Survival outcome; Transformation Risk.

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