Plasmablastic lymphoma(PBL),an extremely rare subtype of B-cell non-Hodgkin lymphoma, is characterized by aggressiveness, rapid progression and bleak prognosis. Neither standardized regimen nor consensus for PBL treatment has been established.

Material and methods:
We retrospectively analyzed the clinicopathologic characteristics, therapeutic modalities and survival outcomes of 418 patients registered in the Surveillance, Epidemiology, and End Results (SEER) database from 2008 to 2016 and 21 (19 treated) patients in our institution. Kaplan-Meier survival curves and log-rank test for overall survival and disease-specific survival were performed to compare each variable. Variables with statistical significance in the univariate Cox regression were incorporated into the multivariate model to determine the independent prognostic factors.

In patient cohort from SEER, PBL has a striking male predilection. The median OS for all PBL patients was 17 months. The 1-year,3-year and 5-year OS rate were 54.4%, 40.4% and 37.2% respectively.Chemotherapy alone or chemotherapy combined with radiotherapy could significantly reduce the risk of death and extend the patients’ survival, yielding HR of 0.209(95%CI 0.152-0.288) and 0.187(95%CI 0.089-0.394), respectively. Radiation alone seemed useless. All patients from our institution were HIV-negative. The main therapeutic regimens were CHOP or CHOPE, DA-EPOCH, DHAP and ESHAP. Complete response was achieved in only 3 patients, while partial response in 10 patients. The median OS was 7 months. Fourteen patients later died of the disease progression.

Previous malignancy history, Ann Arbor stage and therapeutic modality were independent prognostic factors. Bortezomib combined with DA-EPOCH may serve as an effective regimen for PBL. The optimal therapeutic modality necessitates further exploration.

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