Survival analysis in treated plasmablastic lymphoma patients: a population‐based study

Abstract

Herein we analyzed survival outcomes in chemotherapy-treated patients with plasmablastic lymphoma (PBL) diagnosed between 2010 to 2016 (n = 248). Data was acquired from the Surveillance, Epidemiology, and End Results (SEER) 18 registries database (April 2019 release based on November 2018 submission). The majority of patients were male (81.9%) and younger than 60 years (71.0%). Oral and gastrointestinal (GI) sites were the most frequent primary extranodal locations (23% and 19.4%, respectively). Oral primary location was inversely associated with presence of B symptoms and advanced Ann-Arbor stage. The 3-year and 5-year overall survival (OS) rates of treated PBL patients were 54% (95% CI: 46.5%-60.8%) and 52.8% (95% CI: 45.2%-59.8%). Three-year conditional survival for 2-year and 3-year survivors were 90.3% and 97.8%, overlapping the survival of a general population matched by age, sex and calendar year. In a multivariable analysis, oral primary location was associated with not only better OS (HR 0.43; 95% CI: 0.21-0.88, P = .021) but also better lymphoma-specific survival (LSS) (SHR 0.36; 95% CI: 0.15-0.86, P = .022); age ≥60 years was associated with shorter LSS (SHR 1.73; 95% CI: 1.02-2.96, P = .043). Seven registries granted access to HIV status (n = 93) where HIV infection was detected in 52.7% of cases. The HIV status did not affect survival outcomes in unadjusted and adjusted analyses. We identified clinical characteristics associated with survival and showed that treated PBL patients may achieve long-term survival. 1 | INTRODUCTION Plasmablastic lymphoma (PBL) is a rare aggressive non-Hodgkin lymphoma with unknown incidence. Plasmablastic lymphoma was first described as a distinct clinicopathological entity in 1997 by Delecluse et al. and was shown to be associated with HIV infection (15 of 16 cases), Epstein-Barr virus (EBV) infection (9 of 16 cases) and a predilection for oral cavity, particularly jaw mucosa. 1 Subsequently, PBL was described in non-HIV patients with primary involvement outside of the oral cavity and was incorporated as a distinct clinical lymphoma type into the latest World Health Organization lymphoma classification. 2 It is considered to be a subtype of large B-cell lymphoma with plasmablastic morphology and expression of plasma cell markers (CD79a, CD38, CD138 and MUM-1), but lacking expression of B-cell markers (CD20, PAX-5). 3 The differential diagnosis includes immunoblastic diffuse large B-cell lymphoma, ALK positive diffuse large B-cell lymphoma, HHV-8 positive large B-cell lymphoma, primary effusion lymphoma, plasmablastic or anaplastic multiple myeloma and undifferentiated carcinoma. 3-5 Nodal involvement and association with HIV and EBV are typical for PBL but not seen in plasma cell neoplasms. 5-7 Plasmablastic lymphoma is an aggressive lymphoma with overall survival (OS) ranging from 7 to 62 months, according to small Jorge A. Florindez and Juan Pablo Alderuccio contributed equally to this study.

Clinical and Therapeutic Implications of Epstein–Barr Virus in HIV-Related Lymphomas

“…PBL is an uncommon lymphoma with an aggressive clinical course, with a median OS of 6–11 months [ 29 , 107 ]. This lymphoma presents more frequently in men (80%) with a median age of 39–46 years.…”

“…While the prognosis of PBL has improved since the introduction of cART, these patients have a poor outcome, with a median OS of 10–15 months [ 107 , 108 , 129 ]. In this regard, the median CD4+ lymphocyte count at diagnosis is 63–165 cells/µL, indicating a status of important immunodeficiency ( Table 1 ) [ 26 , 131 , 132 ].…”

Light chain cast nephropathy caused by plasmablastic lymphoma of the bladder

“…PBL generally carries a poor prognosis regardless of HIV status, with survival ranging from 7 to 11 months in different risk groups [ 11 ]. More recent data from the Lymphoma Study Association (LYSA) and the Surveillance, Epidemiology, and End Results (SEER) database showed better survival in patients treated with modern chemotherapy regimens, as used in our case [ 26 , 48 ].…”

“…Only one prior case of bladder PBL has been reported to date [ 8 ]. The site of extra-medullary disease does not have a consistent impact on survival or prognosis, although disease limited to oral cavity may have a better prognosis [ 26 ]. Vigilance is required for the development of circulating monoclonal immunoglobulins, as occurred in this case, which can cause kidney injury.…”

Plasmablastic Lymphoma or Plasmablastic Myeloma: A Case of Post-Transplant Lymphoproliferative Disorder

“…Bortezomib and lenalidomide have also been studied, especially in refractory cases [ 9 ]. Though an aggressive disease, treatment with chemotherapy improves survival as reported in a SEER database analysis published in August 2020 which showed a 5-year overall survival of 52.8% in treated PBL patients [ 10 ]. The presence of a MYC rearrangement has been reported to have a poor prognosis [ 7 ].…”