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.
边缘区淋巴瘤是一种惰性B细胞恶性肿瘤，其解剖部位和临床表现具有异质性。虽然该疾病通常与长期生存相关，但部分患者会发生向侵袭性大B细胞淋巴瘤的组织学转化。目前关于边缘区淋巴瘤转化的基于人群的长期数据较为有限。我们在芬兰开展了一项全国性人群研究，旨在评估1995年至2018年间诊断为边缘区淋巴瘤患者的转化风险及相对生存率。通过芬兰癌症登记处共确认1454例边缘区淋巴瘤患者。10年累积转化率为4.7%（95% CI，3.6-6.2）。脾边缘区淋巴瘤患者的转化率最高（14.0%；95% CI，8.6-22.7）。组织学转化与死亡风险显著增加相关（HR，5.18；95% CI，3.58-7.50）。十年相对生存率为79%（95% CI，73-83%）。组织学转化、淋巴结边缘区淋巴瘤亚型及较高诊断年龄与超额死亡风险增加相关，而较晚历法时期确诊的患者超额死亡风险较低。我们的结论是：与未发生转化的患者相比，无论何种亚型，组织学转化均导致死亡率显著上升。研究结果同时提示近年超额死亡率有所下降。
Transformation and survival in marginal zone lymphoma: a Finnish nationwide population-based study
肿瘤(癌症)患者之家