Original Article

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Blood Res 2020; 55(1):

Published online March 31, 2020

https://doi.org/10.5045/br.2020.55.1.49

© The Korean Society of Hematology

HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases

Ya-Jun Li1,2#, Ji-Wei Li3,4,5#, Kai-Lin Chen2,6#, Jin Li1,2, Mei-Zuo Zhong7, Xian-Ling Liu8, Ping-Yong Yi1,2, Hui Zhou1,2

Department of Lymphoma and Hematology, 1Hunan Cancer Hospital, 2The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, 3Department of Pathology, Fudan University Shanghai Cancer Center, 4Department of Oncology, Shanghai Medical College, Fudan University, 5Institute of Pathology, Fudan University, Shanghai, 6Department of Radiotherapy, Key Laboratory of Translational Radiation Oncology, Hunan Cancer Hospital, 7Department of Oncology, Xiangya Hospital, Central South University, 8Department of Oncology, Cancer Center of the Second Xiangya Hospital, Central South University, Changsha, China

Correspondence to : Hui Zhou, M.D.
Department of Lymphoma and Hematology, Hunan Cancer Hospital, 283 Tongzipo Road, Yuelu District, Changsha 410013, China
E-mail: zhouhui@hnca.org.cn

#These authors contributed equally to this work.

Received: November 5, 2019; Revised: February 6, 2020; Accepted: March 7, 2020

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background
Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma (PBL) is a rare entity of diffuse large B-cell lymphoma (DLBCL). The clinicopathological features of and optimal treatment for HIV-negative PBL remain largely unknown.
Methods
To gain insight into this distinct lymphoma, we summarized the clinicopathologic characteristics of 8 unpublished HIV-negative PBLs and performed a comprehensive review of 394 published cases.
Results
Of the 8 unpublished PBLs, the median patient age was 53.0 years. Four patients presented with stage IV disease. All 8 patients showed a plasma cell-like immunophenotype. Of the six patients who received anthracycline-based chemotherapy, including two who received bortezomib, three patients achieved a continuous complete response, two patients died due to disease progression, and one patient was lost to follow-up. The other two patients achieved continuous complete response after receiving chemotherapy combined with radiotherapy and surgery. Of the 402 patients, the majority were male, with a mean age of 58.0 years. EBV infection was detected in 55.7% of the patients. The median survival times of the patients who received CHOP or CHOP-like regimens and intensive regimens were not reached and 23.0 months, respectively, and the intensive regimen did not improve the survival outcome (P=0.981). Multivariate analysis showed that EBER remained the only independent factor affecting overall survival (OS).
Conclusion
HIV-negative PBL is a distinct entity with a predilection for elderly and immunosuppressed individuals. Intensive chemotherapy had no apparent survival benefits over the CHOP regimen in terms of OS; the prognosis of this disease is poor with current chemotherapy methods, and treatment remains a challenge.

Keywords Clinicopathological features, Plasmablastic lymphoma, HIV negative, Treatment, Prognosis

Article

Original Article

Blood Res 2020; 55(1): 49-56

Published online March 31, 2020 https://doi.org/10.5045/br.2020.55.1.49

Copyright © The Korean Society of Hematology.

HIV-negative plasmablastic lymphoma: report of 8 cases and a comprehensive review of 394 published cases

Ya-Jun Li1,2#, Ji-Wei Li3,4,5#, Kai-Lin Chen2,6#, Jin Li1,2, Mei-Zuo Zhong7, Xian-Ling Liu8, Ping-Yong Yi1,2, Hui Zhou1,2

Department of Lymphoma and Hematology, 1Hunan Cancer Hospital, 2The Affiliated Cancer Hospital of Xiangya School of Medicine, Central South University, Changsha, 3Department of Pathology, Fudan University Shanghai Cancer Center, 4Department of Oncology, Shanghai Medical College, Fudan University, 5Institute of Pathology, Fudan University, Shanghai, 6Department of Radiotherapy, Key Laboratory of Translational Radiation Oncology, Hunan Cancer Hospital, 7Department of Oncology, Xiangya Hospital, Central South University, 8Department of Oncology, Cancer Center of the Second Xiangya Hospital, Central South University, Changsha, China

Correspondence to:Hui Zhou, M.D.
Department of Lymphoma and Hematology, Hunan Cancer Hospital, 283 Tongzipo Road, Yuelu District, Changsha 410013, China
E-mail: zhouhui@hnca.org.cn

#These authors contributed equally to this work.

Received: November 5, 2019; Revised: February 6, 2020; Accepted: March 7, 2020

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background
Human immunodeficiency virus (HIV)-negative plasmablastic lymphoma (PBL) is a rare entity of diffuse large B-cell lymphoma (DLBCL). The clinicopathological features of and optimal treatment for HIV-negative PBL remain largely unknown.
Methods
To gain insight into this distinct lymphoma, we summarized the clinicopathologic characteristics of 8 unpublished HIV-negative PBLs and performed a comprehensive review of 394 published cases.
Results
Of the 8 unpublished PBLs, the median patient age was 53.0 years. Four patients presented with stage IV disease. All 8 patients showed a plasma cell-like immunophenotype. Of the six patients who received anthracycline-based chemotherapy, including two who received bortezomib, three patients achieved a continuous complete response, two patients died due to disease progression, and one patient was lost to follow-up. The other two patients achieved continuous complete response after receiving chemotherapy combined with radiotherapy and surgery. Of the 402 patients, the majority were male, with a mean age of 58.0 years. EBV infection was detected in 55.7% of the patients. The median survival times of the patients who received CHOP or CHOP-like regimens and intensive regimens were not reached and 23.0 months, respectively, and the intensive regimen did not improve the survival outcome (P=0.981). Multivariate analysis showed that EBER remained the only independent factor affecting overall survival (OS).
Conclusion
HIV-negative PBL is a distinct entity with a predilection for elderly and immunosuppressed individuals. Intensive chemotherapy had no apparent survival benefits over the CHOP regimen in terms of OS; the prognosis of this disease is poor with current chemotherapy methods, and treatment remains a challenge.

Keywords: Clinicopathological features, Plasmablastic lymphoma, HIV negative, Treatment, Prognosis

Fig 1.

Figure 1.(A) The 1-year and 2-year overall survival rates were 55.9% and 44.8%, respectively (N=253). (B) The median survival time of patients receiving intensive regimens was 23.0 months, while the median survival time of those who received CHOP or CHOP-like regimen has not yet been reached. Intensive regimen did not improve the survival outcome (P=0.981). (C) Significant impact of EBV on overall survival (OS). Patients with EBV positivity showed a significantly better OS (1-year OS, 65.0% vs. 51.5%; P=0.004). (D) Significant impact of MYC rearrangement on OS. MYC rearrangement did not affect the survival outcome (1-year OS, 64.3% vs. 52.4%; P=0.729).
Blood Research 2020; 55: 49-56https://doi.org/10.5045/br.2020.55.1.49

Clinical data of the 8 HIV-negative PBL patients..

Abbreviations: BM involvement, bone marrow involvement; Bor, bortezomib; CHOP, cyclophosphamide+doxorubicin+vincristine+ prednisolone; CHOPE, cyclophosphamide+doxorubicin+vincristine+prednisolone+etoposide; CR, complete remission; CSF cytology, cerebrospinal fluid cytology; DOD, death of disease; ECOG PS, Eastern Cooperative Oncology Group performance status; GEMOX, gemcitabine+oxaliplatin; IFRT, involved-field radiotherapy; IgA, immunoglobulin A; M protein, monoclonal protein; N/A, not applicable; PBL, plasmablastic lymphoma; PD, progressive disease; PR, partial remission..


The immunohistochemical characteristics of the 8 cases of HIV negative PBL cases..

Abbreviations: N/A, not applicable; PBL, plasmablastic lymphoma..


The characteristics of the 402 PBL patients..

Abbreviations: BM, bone marrow; Bor, bortezomib; IC, immunocompetent; LDH, lactate dehydrogenase; LN, lymph node; NA, not available; NR, not reached; PBL, plasmablastic lymphoma; PT, posttransplant..


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