Primary breast double-hit lymphoma management and outcomes: a real-world multicentre experience

Abstract Background Primary breast double-hit lymphoma (PB-DHL) is a rare, highly aggressive malignancy that poses challenges regarding accurate diagnosis and selecting optimal treatment regimens. Methods We retrospectively reviewed 48 cases of patients diagnosed with PB-DHL in six academic centres between June 2014 2020 China. Study-specific data were recorded, including options, therapeutic evaluation, prognostic factors relapse patterns, the overall survival (OS) progression-free (PFS) evaluated. Results In total, enrolled, 14 treated DA-EPOCH-R/MA (rituximab, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, alternating high-dose methotrexate cytarabine), 18 DA-EPOCH-R doxorubicin), 16 R-HyperCVAD hyperfractionated dexamethasone, cytarabine plus methotrexate). The 5-year OS PFS rates 41.7% (95% confidence interval [CI], 27.6–56.8%) 37.5% CI, 24.0–52.6%), respectively. Of three regimens, was higher group than or subgroups (57.1% vs. 38.9% 31.3%; P = 0.016), as (50.0% 25.0%; 0.035). Autologous stem cell transplantation (ASCT) prolonged compared non-ASCT (5-year OS: 72.2% 23.3%; < 0.001; PFS: 16.7 %, 0.001). Multivariate analysis identified tumour size, risk stratification, DA-EPOCH-R/MA, irradiation, ASCT significant factors. Conclusions promising regimen for PB-DHL, irradiation yields complementary benefits prognosis. significantly decreased disease relapse, providing potential curative intervention justifying first-line therapy young patients. More effective strategies remain encouraging.