Abstract
IntroductionPlasmablastic 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 methodsWe 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.ResultsIn 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.ConclusionsPrevious 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.
Highlights
Plasmablastic lymphoma(PBL), an extremely rare subtype of B-cell non-Hodgkin lymphoma(NHL), is characterized by aggressiveness, rapid progression and bleak prognosis
Bortezomib combined with DA-EPOCH may serve as an t effective regimen for PBL
PBL was once regarded as a tumor that predominantly arose in e patients with Human immunodeficiency virus (HIV)-infection[8]
Summary
Plasmablastic lymphoma(PBL) , an extremely rare subtype of B-cell non-Hodgkin lymphoma(NHL), is characterized by aggressiveness, rapid progression and bleak prognosis. Plasmablastic lymphoma (PBL) is an extremely rare and distinct subtype of B-cell non-Hodgkin lymphoma (NHL)[1, 2]. PBL exhibits mixed morphological characteristics of diffuse large B-cell lymphoma (DLBCL) and multiple myeloma (MM) with its highly aggressive attribute and plasmacytic differentiation[3]. The cell origin of PBL is considered the post-germinal center B-lymphocyte or plasmablast. It is often believed that PBL afflicts immunocompromised individuals, including those infected with human in immunodeficiency virus(HIV), those receiving intense chemotherapy or radiotherapy for r cancers, those undergoing organ or stem cell transplantation and those receiving p immunorepressive drugs[5-7]. Castillo et al reported 71 HIV-negative PBL r patients with unique clinicopathological characteristics markedly distinct from their P HIV-positive counterparts[9]
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