Chen Guang-Liang, Li Doudou, Cao Sufen, Jiang Shiyu, Zhang Qunling, Jin Jia, Xia Zuguang, Liu Yizhen, Liu Xiaojian, Zhu Yanzhe, Chen Yu, Gu Lingli, Hong Xiaonan, Cao Junning, Tao Rong, Lv Fangfang
Department of Lymphoma, Fudan University Shanghai Cancer Center, Shanghai, 200032, China.
Department of Oncology, Shanghai Medical College Fudan University, Shanghai, 200032, China.
Mediterr J Hematol Infect Dis. 2022 Sep 1;14(1):e2022066. doi: 10.4084/MJHID.2022.066. eCollection 2022.
Primary breast diffuse large B-cell lymphoma (PB-DLBCL) is a rare subtype of non-Hodgkin lymphoma (NHL) with limited data on the clinical features and prognostic factors.
A consecutive cohort of patients with PB-DLBCL was retrospectively analyzed in our hospital from February 1997 through July 2018. The primary endpoint is overall survival (OS) contributing to any cause.
A total of 76 patients were diagnosed with PB-DLBCL. The median age at diagnosis was 51 years (range: 25-80 years), with female prevalence (98.7%). Forty (52.6%) patients had right-sided breast involvement but no bilateral breast involvement at diagnosis. Overall, disease stages IE and IIE were seen in 55 (72.4%) and 21 (27.6%) patients, respectively. According to the stage-modified International Prognostic Index (IPI), 37 (48.7%) patients were classified in the very good risk group (IPI 0). Of the 72 patients available, the non-germinal center B-cell (non-GCB) subtype of DLBCL was observed in 66 (91.6%) patients. All patients received anthracycline-based chemotherapy, 56 (73.7%) with rituximab, 31 (40.8%) also with additional radiation therapy, and 14 (18.4%) patients received a prophylactic intrathecal injection. Seven (9.2%) patients had refractory disease. With a median follow-up of 6.8 years (range 0.4-25.0 years), 10 (13.2%) patients had a relapse in the central nervous system (CNS) site. The 5-year and 10-year OS of all the patients was 97.2% (95% CI: 99.3-89.5) and 84.8% (95% CI: 70.0-93.5), respectively. The median OS was not reached. The median progression-free survival (PFS) was 10.3 years for patients with PB-DLBCL. The 5-year PFS of all the patients was 76.3% (95% CI: 64.6-84.6). Univariate analysis revealed several prognostic factors, including stage-modified IPI, breast surgery, refractory disease, and CNS relapse. Multivariate analyses produced two independent prognostic factors for patients with PB-DLBCL, including stage-modified IPI score (2-3 versus 0) (hazard ratio: 19.114, 95% CI 1.841 to 198.451, p=0.013) and CNS relapse (hazard ratio: 5.522, 95% CI 1.059 to 28.788, p=0.043).
In our cohort, PB-DLBCL clinical features are similar to prior literature reports. Stage-modified IPI score and CNS relapse were associated with overall survival.
原发性乳腺弥漫性大B细胞淋巴瘤（PB-DLBCL）是一种罕见的非霍奇金淋巴瘤（NHL）亚型，关于其临床特征和预后因素的数据有限。
回顾性分析了1997年2月至2018年7月在我院连续收治的PB-DLBCL患者队列。主要终点是任何原因导致的总生存期（OS）。
共76例患者被诊断为PB-DLBCL。诊断时的中位年龄为51岁（范围：25 - 80岁），女性占比（98.7%）。40例（52.6%）患者诊断时右侧乳腺受累，但无双侧乳腺受累。总体而言，疾病分期为IE期和IIE期的患者分别有55例（72.4%）和21例（27.6%）。根据分期修正的国际预后指数（IPI），37例（48.7%）患者被归类为极低风险组（IPI 0）。在72例可评估患者中，66例（91.6%）患者为弥漫性大B细胞淋巴瘤（DLBCL）的非生发中心B细胞（non-GCB）亚型。所有患者均接受了以蒽环类为基础的化疗，56例（73.7%）联合利妥昔单抗，31例（40.8%）还接受了额外的放射治疗，14例（18.4%）患者接受了预防性鞘内注射。7例（9.2%）患者疾病难治。中位随访6.8年（范围0.4 - 25.0年），10例（13.2%）患者出现中枢神经系统（CNS）部位复发。所有患者的5年和10年总生存率分别为97.2%（95%CI：99.3 - 89.5）和84.8%（95%CI：70.0 - 93.5）。中位总生存期未达到。PB-DLBCL患者的中位无进展生存期（PFS）为10.3年。所有患者的5年无进展生存率为76.3%（95%CI：64.6 - 84.6）。单因素分析显示了几个预后因素，包括分期修正的IPI、乳腺手术、难治性疾病和中枢神经系统复发。多因素分析得出PB-DLBCL患者的两个独立预后因素，包括分期修正的IPI评分（2 - 3对比0）（风险比：19.114，95%CI 1.841至198.451，p = 0.013）和中枢神经系统复发（风险比：5.522，95%CI 1.059至28.788，p = 0.043）。
在我们的队列中，PB-DLBCL的临床特征与既往文献报道相似。分期修正的IPI评分和中枢神经系统复发与总生存期相关。
