Clinicopathologic characteristics, therapeutic modalities and survival outcomes of plasmablastic lymphoma: a real-world study.

INTRODUCTION

Plasmablastic lymphoma (PBL), an extremely rare subtype of B-cell non-Hodgkin lymphoma (NHL), is characterized by aggressiveness, rapid progression and a bleak prognosis. Neither a standardized regimen nor a 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 the log-rank test for overall survival (OS) and disease-specific survival (DSS) were performed to compare each variable. Variables with statistical significance in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors.

RESULTS

In the patient cohort from the SEER database, 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 rates were 54.4%, 40.4% and 37.2% respectively. Patients who suffered from previous malignancy had a significant survival disadvantage compared to those without previous cancer. Patients with a higher Ann Arbor stage at diagnosis were at higher risk of death than those with a lower stage. Chemotherapy alone or chemotherapy combined with radiotherapy could significantly reduce the risk of death and extend the patients' survival, yielding a 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. A complete response (CR) was achieved in only 3 patients, while a partial response was achieved in 10 patients. The median OS was 7 months. Fourteen patients later died due to disease progression.

CONCLUSIONS

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.