https://orcid.org/0000-0002-9282-8619
PBL is a rare non-Hodgkin lymphoma with a poor prognosis and uncertainty regarding optimal treatment approaches.
Advanced stage, bone marrow involvement, ECOG > 1, and EBV–negative lymphoma are associated with inferior OS.
Plasmablastic lymphoma (PBL) is a rare and aggressive non-Hodgkin lymphoma associated with immunodeficiency, characterized by uncertain treatment approaches and an unfavorable prognosis. We conducted a multicenter, international, retrospective cohort study, aiming to characterize the clinical features, risk factors, and outcomes of patients with PBL. Data were collected from 22 institutions across 4 countries regarding patients diagnosed with PBL between 1 January 1999 and 31 December 2020. Survival risk factors were analyzed using both univariate and multivariate regression models. Overall survival (OS) was calculated using Kaplan-Meier statistics. First-line treatment regimens were stratified into standard- and higher-intensity regimens, and based on whether they incorporated a proteasome inhibitor (PI). A total of 281 patients (median age, 55 years) were included. Immunodeficiency of any kind was identified in 144 patients (51%), and 99 patients (35%) had HIV-positive results. The 5-year OS for the entire cohort was 36% (95% confidence interval, 30%-42%). In multivariate analysis, inferior OS was associated with Epstein-Barr virus–negative lymphoma, poor performance status, advanced stage, and bone marrow involvement. In an independent univariate analysis, the international prognostic index was associated with OS outcomes. Neither immunosuppression nor HIV infection, specifically, influenced OS. Among patients treated with curative intent (n = 234), the overall response rate was 72%. Neither the intensity of the treatment regimen nor the inclusion of PIs in first-line therapy was associated with OS. In this large retrospective study of patients with PBL, we identified novel risk factors for survival. PBL remains a challenging disease with poor long-term outcomes.
Comments
Response to Dr. Venkataraman
Similarly for CNS involvement, the p-value provided for in Table 1 is a test of equality of proportions and not two-way pair wise comparison, as there are more than two categories (“not-tested/unknown” values were included). PI-containing regimens were not reported as significantly different amongst IS and NIS patients in our analysis.
The proportion of oropharyngeal tumor location among HIV positive patients was 29% versus 31% amongst HIV negative patients.
CD20