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.
边缘区淋巴瘤（MZL）向弥漫性大B细胞淋巴瘤的组织学转化（tMZL）与不良预后相关。由于数据稀缺，与转化风险及转化后结局相关的临床特征在很大程度上尚未明确。这项基于人群的研究利用荷兰癌症登记处的1793例MZL患者数据，通过竞争风险分析阐明与发生转化相关的临床特征，并采用Cox回归分析揭示与转化后复发风险和死亡率相关的临床特征。在1793例MZL患者中，75例（4%）发生了转化。MZL诊断时LDH升高和淋巴结MZL亚型与转化风险增加相关，而放疗可降低tMZL发生风险。大多数tMZL患者接受了R-(mini)CHOP方案治疗（n=53，71%）。年龄＞60岁以及转化前接受过（免疫）化疗与转化后复发风险和死亡率增加相关。接受R-(mini)CHOP治疗的tMZL患者2年无进展生存期（PFS）和总生存期（OS）分别为66%（95% CI 52–77%）和75%（95% CI 62–85%），而接受其他治疗方案患者的PFS和OS均为41%（95% CI 19–63%）。本研究全面揭示了与淋巴瘤转化及转化后生存相关的特征，从而为优化临床指南和患者咨询提供了依据。
肿瘤(癌症)患者之家