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@article{Di:2024:10.1182/blood.2023021348,
author = {Di, Ciaccio PR and Polizzotto, MN and Cwynarski, K and Gerrie, AS and Burton, C and Bower, M and Kuruvilla, J and Montoto, S and McKay, P and Fox, CP and Milliken, S and Jiamsakul, A and Osborne, W and Collins, GP and Manos, K and Linton, KM and Iyengar, S and Kassam, S and Limei, MP and Kliman, D and Wong, Doo N and Watson, A-M and Fedele, P and Yannakou, CK and Hunt, S and Ku, M and Sehn, LH and Smith, A and Renshaw, H and Maxwell, A and Liu, Q and Dhairyawan, R and Ferguson, G and Pickard, K and Painter, D and Thakrar, N and Song, KW and Hamad, N},
doi = {10.1182/blood.2023021348},
journal = {Blood},
pages = {152--165},
title = {The influence of immunodeficiency, disease features, and patient characteristics on survival in plasmablastic lymphoma.},
url = {http://dx.doi.org/10.1182/blood.2023021348},
volume = {143},
year = {2024}
}

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TY  - JOUR
AB  - 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.
AU  - Di,Ciaccio PR
AU  - Polizzotto,MN
AU  - Cwynarski,K
AU  - Gerrie,AS
AU  - Burton,C
AU  - Bower,M
AU  - Kuruvilla,J
AU  - Montoto,S
AU  - McKay,P
AU  - Fox,CP
AU  - Milliken,S
AU  - Jiamsakul,A
AU  - Osborne,W
AU  - Collins,GP
AU  - Manos,K
AU  - Linton,KM
AU  - Iyengar,S
AU  - Kassam,S
AU  - Limei,MP
AU  - Kliman,D
AU  - Wong,Doo N
AU  - Watson,A-M
AU  - Fedele,P
AU  - Yannakou,CK
AU  - Hunt,S
AU  - Ku,M
AU  - Sehn,LH
AU  - Smith,A
AU  - Renshaw,H
AU  - Maxwell,A
AU  - Liu,Q
AU  - Dhairyawan,R
AU  - Ferguson,G
AU  - Pickard,K
AU  - Painter,D
AU  - Thakrar,N
AU  - Song,KW
AU  - Hamad,N
DO  - 10.1182/blood.2023021348
EP  - 165
PY  - 2024///
SP  - 152
TI  - The influence of immunodeficiency, disease features, and patient characteristics on survival in plasmablastic lymphoma.
T2  - Blood
UR  - http://dx.doi.org/10.1182/blood.2023021348
UR  - https://www.ncbi.nlm.nih.gov/pubmed/37832030
VL  - 143
ER  -

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