Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China

Abstract Background Plasmablastic lymphoma (PBL) is a rare but aggressive B-cell lymphoma subtype with poor prognosis. Knowledge about the etiology, clinicopathologic and molecular features, and outcomes of PBL is limited. This study aimed to examine the clinicopathologic characteristics, therapeuti...

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Main Authors: Di Shi, Lin Gao, Xiao-Chun Wan, Jin Li, Tian Tian, Jue Hu, Qun-Ling Zhang, Yi-Fan Su, Yu-Peng Zeng, Zi-Juan Hu, Bao-Hua Yu, Xiao-Qiu Li, Ping Wei, Ji-Wei Li, Xiao-Yan Zhou
Format: Article
Language:English
Published: BMC 2022-12-01
Series:BMC Medicine
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Online Access:https://doi.org/10.1186/s12916-022-02683-9
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author Di Shi
Lin Gao
Xiao-Chun Wan
Jin Li
Tian Tian
Jue Hu
Qun-Ling Zhang
Yi-Fan Su
Yu-Peng Zeng
Zi-Juan Hu
Bao-Hua Yu
Xiao-Qiu Li
Ping Wei
Ji-Wei Li
Xiao-Yan Zhou
author_facet Di Shi
Lin Gao
Xiao-Chun Wan
Jin Li
Tian Tian
Jue Hu
Qun-Ling Zhang
Yi-Fan Su
Yu-Peng Zeng
Zi-Juan Hu
Bao-Hua Yu
Xiao-Qiu Li
Ping Wei
Ji-Wei Li
Xiao-Yan Zhou
author_sort Di Shi
collection DOAJ
description Abstract Background Plasmablastic lymphoma (PBL) is a rare but aggressive B-cell lymphoma subtype with poor prognosis. Knowledge about the etiology, clinicopathologic and molecular features, and outcomes of PBL is limited. This study aimed to examine the clinicopathologic characteristics, therapeutic approaches, and clinical outcomes of PBL patients in a Chinese population. Methods A total of 102 PBL patients were recruited from three cancer centers. The pathologic features and clinical outcomes of 56 patients with available treatment details and follow-up data were reviewed and analyzed. RNA sequencing was performed in 6 PBL and 11 diffuse large B-cell lymphoma (DLBCL) patients. Results Most patients in our cohort were male (n = 36, 64.3%), and 35 patients presented with Ann Arbor stage I/II disease at diagnosis. All these patients showed negative findings for human immunodeficiency virus, and the vast majority of patients in our cohort were immunocompetent. Lymph nodes (n = 13, 23.2%) and gastrointestinal tract (n = 10, 17.9%) were the most commonly involved site at presentation. Post-treatment complete remission (CR) was the only prognostic factor affecting overall survival (OS) and progression-free survival (PFS) in the multivariate analysis. RNA-seq demonstrated that B-cell receptor (BCR), T-cell receptor (TCR), P53, calcium signaling, and Wnt signaling pathways were significantly downregulated in PBLs compared with GCB (or non-GCB) DLBCLs. Conclusions In this multicenter study in the Chinese population, PBL mainly occurred in immunocompetent individuals and most patients present with early-stage disease at diagnosis. Post-treatment CR was an important prognostic factor affecting OS and PFS. RNA-seq showed that the B-cell receptor (BCR), P53, calcium signaling, cell adhesion molecules, and Wnt signaling pathways significantly differed between PBL and GCB (or non-GCB) DLBCL, which provided theoretical basis for its pathogenesis and future treatment.
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spelling doaj.art-29e1c29f8f2a4010985723e7e45fc9182022-12-22T04:23:30ZengBMCBMC Medicine1741-70152022-12-0120111010.1186/s12916-022-02683-9Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in ChinaDi Shi0Lin Gao1Xiao-Chun Wan2Jin Li3Tian Tian4Jue Hu5Qun-Ling Zhang6Yi-Fan Su7Yu-Peng Zeng8Zi-Juan Hu9Bao-Hua Yu10Xiao-Qiu Li11Ping Wei12Ji-Wei Li13Xiao-Yan Zhou14Department of Pathology, Fudan University Shanghai Cancer CenterGenePlus-ShenzhenDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Oncology, Hunan Cancer HospitalDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Pathology, Fudan University Shanghai Cancer CenterDepartment of Oncology, The Second Xiangya Hospital, Central South UniversityDepartment of Pathology, Fudan University Shanghai Cancer CenterAbstract Background Plasmablastic lymphoma (PBL) is a rare but aggressive B-cell lymphoma subtype with poor prognosis. Knowledge about the etiology, clinicopathologic and molecular features, and outcomes of PBL is limited. This study aimed to examine the clinicopathologic characteristics, therapeutic approaches, and clinical outcomes of PBL patients in a Chinese population. Methods A total of 102 PBL patients were recruited from three cancer centers. The pathologic features and clinical outcomes of 56 patients with available treatment details and follow-up data were reviewed and analyzed. RNA sequencing was performed in 6 PBL and 11 diffuse large B-cell lymphoma (DLBCL) patients. Results Most patients in our cohort were male (n = 36, 64.3%), and 35 patients presented with Ann Arbor stage I/II disease at diagnosis. All these patients showed negative findings for human immunodeficiency virus, and the vast majority of patients in our cohort were immunocompetent. Lymph nodes (n = 13, 23.2%) and gastrointestinal tract (n = 10, 17.9%) were the most commonly involved site at presentation. Post-treatment complete remission (CR) was the only prognostic factor affecting overall survival (OS) and progression-free survival (PFS) in the multivariate analysis. RNA-seq demonstrated that B-cell receptor (BCR), T-cell receptor (TCR), P53, calcium signaling, and Wnt signaling pathways were significantly downregulated in PBLs compared with GCB (or non-GCB) DLBCLs. Conclusions In this multicenter study in the Chinese population, PBL mainly occurred in immunocompetent individuals and most patients present with early-stage disease at diagnosis. Post-treatment CR was an important prognostic factor affecting OS and PFS. RNA-seq showed that the B-cell receptor (BCR), P53, calcium signaling, cell adhesion molecules, and Wnt signaling pathways significantly differed between PBL and GCB (or non-GCB) DLBCL, which provided theoretical basis for its pathogenesis and future treatment.https://doi.org/10.1186/s12916-022-02683-9Plasmablastic lymphomaImmunocompetentRNA-sequencing
spellingShingle Di Shi
Lin Gao
Xiao-Chun Wan
Jin Li
Tian Tian
Jue Hu
Qun-Ling Zhang
Yi-Fan Su
Yu-Peng Zeng
Zi-Juan Hu
Bao-Hua Yu
Xiao-Qiu Li
Ping Wei
Ji-Wei Li
Xiao-Yan Zhou
Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
BMC Medicine
Plasmablastic lymphoma
Immunocompetent
RNA-sequencing
title Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
title_full Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
title_fullStr Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
title_full_unstemmed Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
title_short Clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma: a multicenter study in China
title_sort clinicopathologic features and abnormal signaling pathways in plasmablastic lymphoma a multicenter study in china
topic Plasmablastic lymphoma
Immunocompetent
RNA-sequencing
url https://doi.org/10.1186/s12916-022-02683-9
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