The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma

Pietro R Di Ciaccio; Mark N Polizzotto; Kate Cwynarski; Alina S Gerrie; Catherine Burton; Mark Bower; John Kuruvilla; Silvia Montoto; Pamela McKay; Christopher P Fox; Samuel Milliken; Awachana Jiamsakul; Wendy Osborne; Graham P Collins; Kate Manos; Kim M Linton; Sunil Iyengar; Shireen Kassam; Michelle Poon Limei; David S Kliman; Nicole Wong Doo; Anne-Marie Watson; Pasquale Fedele; Costas K Yannakou; Stewart Hunt; Matthew Ku; Laurie H Sehn; Alexandra Smith; Hanna Renshaw; Alice Maxwell; Qin Liu; Rageshri Dhairyawan; Graeme Ferguson; Keir Pickard; Daniel Painter; Nisha Thakrar; Kevin W Song; Nada Hamad

doi:10.1182/blood.2023021348

The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma

Pietro R Di Ciaccio, Mark N Polizzotto, Kate Cwynarski, Alina S Gerrie, Catherine Burton, Mark Bower, John Kuruvilla, Silvia Montoto, Pamela McKay, Christopher P Fox, Samuel Milliken, Awachana Jiamsakul, Wendy Osborne, Graham P Collins, Kate Manos, Kim M Linton, Sunil Iyengar, Shireen Kassam, Michelle Poon Limei, David S KlimanNicole Wong Doo, Anne-Marie Watson, Pasquale Fedele, Costas K Yannakou, Stewart Hunt, Matthew Ku, Laurie H Sehn, Alexandra Smith, Hanna Renshaw, Alice Maxwell, Qin Liu, Rageshri Dhairyawan, Graeme Ferguson, Keir Pickard, Daniel Painter, Nisha Thakrar, Kevin W Song, Nada Hamad

Health Sciences

Research output: Contribution to journal › Article › peer-review

Abstract

Plasmablastic lymphoma (PBL) is a rare and aggressive non-Hodgkin lymphoma associated with immunodeficiency, characterized by uncertain treatment approaches and an unfavourable 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 four 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 by whether they incorporated a proteasome inhibitor (PI). A total of 281 patients (median age 55) were included. Immunodeficiency of any kind was identified in 144 patients (51%), and 99 patients (35%) were HIV-positive. The five-year OS for the entire cohort was 36% (95% CI 30-42%). In multivariate analysis, inferior OS was associated with EBV-negative lymphoma, poor performance status, advanced stage, and bone marrow involvement. In an independent univariate analysis, the IPI 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 PBL patients, we identified novel risk factors for survival. PBL remains a challenging disease with poor long-term outcomes.

Original language	English
Journal	Blood
Early online date	13 Oct 2023
DOIs	https://doi.org/10.1182/blood.2023021348
Publication status	E-pub ahead of print - 13 Oct 2023

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10.1182/blood.2023021348

1-s2.0-S0006497123025491-main
© 2023 American Society of Hematology. This is an author-produced version of the published paper. Uploaded in accordance with the University’s Research Publications and Open Access policy.
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Cite this

Di Ciaccio, P. R., Polizzotto, M. N., Cwynarski, K., Gerrie, A. S., Burton, C., Bower, M., Kuruvilla, J., Montoto, S., McKay, P., Fox, C. P., Milliken, S., Jiamsakul, A., Osborne, W., Collins, G. P., Manos, K., Linton, K. M., Iyengar, S., Kassam, S., Poon Limei, M., ... Hamad, N. (2023). The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma. Blood. Advance online publication. https://doi.org/10.1182/blood.2023021348

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title = "The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma",

abstract = "Plasmablastic lymphoma (PBL) is a rare and aggressive non-Hodgkin lymphoma associated with immunodeficiency, characterized by uncertain treatment approaches and an unfavourable 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 four 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 by whether they incorporated a proteasome inhibitor (PI). A total of 281 patients (median age 55) were included. Immunodeficiency of any kind was identified in 144 patients (51%), and 99 patients (35%) were HIV-positive. The five-year OS for the entire cohort was 36% (95% CI 30-42%). In multivariate analysis, inferior OS was associated with EBV-negative lymphoma, poor performance status, advanced stage, and bone marrow involvement. In an independent univariate analysis, the IPI 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 PBL patients, we identified novel risk factors for survival. PBL remains a challenging disease with poor long-term outcomes.",

author = "{Di Ciaccio}, {Pietro R} and Polizzotto, {Mark N} and Kate Cwynarski and Gerrie, {Alina S} and Catherine Burton and Mark Bower and John Kuruvilla and Silvia Montoto and Pamela McKay and Fox, {Christopher P} and Samuel Milliken and Awachana Jiamsakul and Wendy Osborne and Collins, {Graham P} and Kate Manos and Linton, {Kim M} and Sunil Iyengar and Shireen Kassam and {Poon Limei}, Michelle and Kliman, {David S} and {Wong Doo}, Nicole and Anne-Marie Watson and Pasquale Fedele and Yannakou, {Costas K} and Stewart Hunt and Matthew Ku and Sehn, {Laurie H} and Alexandra Smith and Hanna Renshaw and Alice Maxwell and Qin Liu and Rageshri Dhairyawan and Graeme Ferguson and Keir Pickard and Daniel Painter and Nisha Thakrar and Song, {Kevin W} and Nada Hamad",

note = "Copyright {\textcopyright} 2023 American Society of Hematology. This is an author-produced version of the published paper. Uploaded in accordance with the University{\textquoteright}s Research Publications and Open Access policy.",

year = "2023",

month = oct,

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Di Ciaccio, PR, Polizzotto, MN, Cwynarski, K, Gerrie, AS, Burton, C, Bower, M, Kuruvilla, J, Montoto, S, McKay, P, Fox, CP, Milliken, S, Jiamsakul, A, Osborne, W, Collins, GP, Manos, K, Linton, KM, Iyengar, S, Kassam, S, Poon Limei, M, Kliman, DS, Wong Doo, N, Watson, A-M, Fedele, P, Yannakou, CK, Hunt, S, Ku, M, Sehn, LH, Smith, A, Renshaw, H, Maxwell, A, Liu, Q, Dhairyawan, R, Ferguson, G, Pickard, K, Painter, D, Thakrar, N, Song, KW & Hamad, N 2023, 'The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma', Blood. https://doi.org/10.1182/blood.2023021348

TY - JOUR

T1 - The Influence of Immunodeficiency, Disease Features and Patient Characteristics on Survival in Plasmablastic Lymphoma

AU - Di Ciaccio, Pietro R

AU - Polizzotto, Mark N

AU - Cwynarski, Kate

AU - Gerrie, Alina S

AU - Burton, Catherine

AU - Bower, Mark

AU - Kuruvilla, John

AU - Montoto, Silvia

AU - McKay, Pamela

AU - Fox, Christopher P

AU - Milliken, Samuel

AU - Jiamsakul, Awachana

AU - Osborne, Wendy

AU - Collins, Graham P

AU - Manos, Kate

AU - Linton, Kim M

AU - Iyengar, Sunil

AU - Kassam, Shireen

AU - Poon Limei, Michelle

AU - Kliman, David S

AU - Wong Doo, Nicole

AU - Watson, Anne-Marie

AU - Fedele, Pasquale

AU - Yannakou, Costas K

AU - Hunt, Stewart

AU - Ku, Matthew

AU - Sehn, Laurie H

AU - Smith, Alexandra

AU - Renshaw, Hanna

AU - Maxwell, Alice

AU - Liu, Qin

AU - Dhairyawan, Rageshri

AU - Ferguson, Graeme

AU - Pickard, Keir

AU - Painter, Daniel

AU - Thakrar, Nisha

AU - Song, Kevin W

AU - Hamad, Nada

N1 - Copyright © 2023 American Society of Hematology. This is an author-produced version of the published paper. Uploaded in accordance with the University’s Research Publications and Open Access policy.

PY - 2023/10/13

Y1 - 2023/10/13

N2 - Plasmablastic lymphoma (PBL) is a rare and aggressive non-Hodgkin lymphoma associated with immunodeficiency, characterized by uncertain treatment approaches and an unfavourable 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 four 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 by whether they incorporated a proteasome inhibitor (PI). A total of 281 patients (median age 55) were included. Immunodeficiency of any kind was identified in 144 patients (51%), and 99 patients (35%) were HIV-positive. The five-year OS for the entire cohort was 36% (95% CI 30-42%). In multivariate analysis, inferior OS was associated with EBV-negative lymphoma, poor performance status, advanced stage, and bone marrow involvement. In an independent univariate analysis, the IPI 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 PBL patients, we identified novel risk factors for survival. PBL remains a challenging disease with poor long-term outcomes.

AB - Plasmablastic lymphoma (PBL) is a rare and aggressive non-Hodgkin lymphoma associated with immunodeficiency, characterized by uncertain treatment approaches and an unfavourable 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 four 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 by whether they incorporated a proteasome inhibitor (PI). A total of 281 patients (median age 55) were included. Immunodeficiency of any kind was identified in 144 patients (51%), and 99 patients (35%) were HIV-positive. The five-year OS for the entire cohort was 36% (95% CI 30-42%). In multivariate analysis, inferior OS was associated with EBV-negative lymphoma, poor performance status, advanced stage, and bone marrow involvement. In an independent univariate analysis, the IPI 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 PBL patients, we identified novel risk factors for survival. PBL remains a challenging disease with poor long-term outcomes.

U2 - 10.1182/blood.2023021348

DO - 10.1182/blood.2023021348

M3 - Article

C2 - 37832030

SN - 0006-4971

JO - Blood

JF - Blood

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