Zhang Tingting, Zhang Yuanfeng, Fei Hairong, Shi Xue, Wang Liang, Wang Peijun, Yu Jie, Shen Yuyan, Feng Sizhou
Haematopoietic Stem Cell Transplantation Centre, State Key Laboratory of Experimental Hematology, National Clinical Research centre for Blood Diseases, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 288 Nanjing Road, Tianjin, 300020, China.
Department of Haematology, The Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, 264000, Shandong, China.
Cancer Cell Int. 2021 Sep 17;21(1):498. doi: 10.1186/s12935-021-02198-y.
Primary breast double-hit lymphoma (PB-DHL) is a rare, highly aggressive malignancy that poses challenges regarding accurate diagnosis and selecting optimal treatment regimens.
We retrospectively reviewed 48 cases of patients diagnosed with PB-DHL in six academic centres between June 2014 and June 2020 in China. Study-specific data were recorded, including treatment options, therapeutic evaluation, prognostic factors and relapse patterns, and the overall survival (OS) and progression-free survival (PFS) were evaluated.
In total, 48 patients were enrolled, with 14 patients treated with DA-EPOCH-R/MA (rituximab, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, alternating with high-dose methotrexate and cytarabine), 18 patients treated with DA-EPOCH-R (rituximab, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin), and 16 patients treated with R-HyperCVAD (rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, alternating with cytarabine plus methotrexate). The overall 5-year OS and PFS rates were 41.7% (95% confidence interval [CI], 27.6-56.8%) and 37.5% (95% CI, 24.0-52.6%), respectively. Of the three treatment regimens, the 5-year OS was higher in DA-EPOCH-R/MA group than in the DA-EPOCH-R or R-HyperCVAD subgroups (57.1% vs. 38.9% vs. 31.3%; P = 0.016), as was the 5-year PFS (50.0% vs. 38.9% vs. 25.0%; P = 0.035). Autologous stem cell transplantation (ASCT) prolonged the OS and PFS compared with non-ASCT patients (5-year OS: 72.2% vs. 23.3%; P < 0.001; 5-year PFS: 72.2% vs. 16.7 %, P < 0.001). Multivariate analysis identified tumour size, risk stratification, treatment with DA-EPOCH-R/MA, breast irradiation, and ASCT as significant prognostic factors.
DA-EPOCH-R/MA is a promising regimen for PB-DHL, and breast irradiation yields complementary benefits for prognosis. ASCT significantly decreased disease relapse, providing a potential curative PB-DHL intervention and justifying ASCT as first-line therapy for young patients. More effective treatment strategies for PB-DHL patients remain encouraging.
原发性乳腺双打击淋巴瘤（PB-DHL）是一种罕见的、侵袭性很强的恶性肿瘤，在准确诊断和选择最佳治疗方案方面存在挑战。
我们回顾性分析了2014年6月至2020年6月期间在中国六个学术中心诊断为PB-DHL的48例患者。记录了特定研究数据，包括治疗方案、治疗评估、预后因素和复发模式，并评估了总生存期（OS）和无进展生存期（PFS）。
共纳入48例患者，14例接受DA-EPOCH-R/MA（利妥昔单抗、剂量调整的依托泊苷、泼尼松、长春新碱、环磷酰胺、多柔比星，交替使用大剂量甲氨蝶呤和阿糖胞苷）治疗，18例接受DA-EPOCH-R（利妥昔单抗、剂量调整的依托泊苷、泼尼松、长春新碱、环磷酰胺、多柔比星）治疗，16例接受R-HyperCVAD（利妥昔单抗、超分割环磷酰胺、长春新碱、多柔比星、地塞米松，交替使用阿糖胞苷加甲氨蝶呤）治疗。5年总生存率和无进展生存率分别为41.7%（95%置信区间[CI]，27.6-56.8%）和37.5%（95%CI，24.0-52.6%）。在三种治疗方案中，DA-EPOCH-R/MA组的5年总生存率高于DA-EPOCH-R或R-HyperCVAD亚组（57.1%对38.9%对31.3%；P = 0.016），5年无进展生存率也是如此（50.0%对38.9%对25.0%；P = 0.035）。与未进行自体干细胞移植（ASCT）的患者相比，ASCT延长了总生存期和无进展生存期（5年总生存率：72.2%对23.3%；P < 0.001；5年无进展生存率：72.2%对16.7%，P < 0.001）。多因素分析确定肿瘤大小、风险分层、DA-EPOCH-R/MA治疗、乳腺放疗和ASCT为显著的预后因素。
DA-EPOCH-R/MA是治疗PB-DHL的一种有前景的方案，乳腺放疗对预后有互补益处。ASCT显著降低了疾病复发率，为PB-DHL提供了一种潜在的治愈性干预措施，证明ASCT可作为年轻患者的一线治疗方法。对于PB-DHL患者，更有效的治疗策略仍令人期待。
