Primary plasmablastic lymphoma of the gastrointestinal tract: A series of 13 HIV-negative cases and a review of literature

Zhiyan Fu; Haibo Wang; Gregory Y Lauwers; Kun Jiang; Nushani L Jayaratne; Sanjay Bridglal; Ning Dong; Endi Wang; Lugen Chen; Deniz Peker Barclift; Ling Zhang

doi:10.1016/j.anndiagpath.2023.152204

Primary plasmablastic lymphoma of the gastrointestinal tract: A series of 13 HIV-negative cases and a review of literature

Ann Diagn Pathol. 2023 Dec:67:152204. doi: 10.1016/j.anndiagpath.2023.152204. Epub 2023 Aug 19.

Authors

Zhiyan Fu¹, Haibo Wang², Gregory Y Lauwers³, Kun Jiang³, Nushani L Jayaratne⁴, Sanjay Bridglal⁴, Ning Dong⁵, Endi Wang⁶, Lugen Chen⁷, Deniz Peker Barclift⁸, Ling Zhang⁹

Affiliations

¹ Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL 33612, USA; Department of Pathology, LSU Health Sciences Center New Orleans, New Orleans, LA 70112, USA.
² Department of Pathology, LSU Health Sciences Center New Orleans, New Orleans, LA 70112, USA.
³ Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL 33612, USA.
⁴ Department of Pathology, University of South Florida Morsani College of Medicine, Tampa, FL 33612, USA.
⁵ Department of Hematological Malignancy, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL 33612, USA.
⁶ Department of Pathology, Duke University, Durham, NC 27710, USA.
⁷ Department of Pathology, Tampa General Hospital, Tampa, FL 33606, USA.
⁸ Department of Pathology, Emory University, Atlanta, GA 30322, USA.
⁹ Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL 33612, USA. Electronic address: ling.zhang@Moffitt.org.

PMID: 37639839
DOI: 10.1016/j.anndiagpath.2023.152204

Abstract

Context: Primary gastrointestinal plasmablastic lymphoma (GI-PBL) is a rare variant of diffuse B-cell lymphoma with an aggressive clinical course. PBL was initially reported among HIV-positive patients; however, subsequent studies have shown that it also occurs among HIV-negative patients. Its clinical characteristics remain poorly understood. This study aims to retrospectively analyze the clinicopathological findings of primary GI-PBLs in HIV-negative patients.

Design: Primary HIV-negative GI-PBL cases from 2008 to 2022 were reviewed. Clinicopathologic features and outcomes were analyzed.

Results: The cohort of 13 patients had a male-to-female ratio of 9:1 (3 patients' genders not available), with an average age of 61 (range, 30-92) years. The most involved location was the colon (n = 7 [53.8 %]), followed by the small bowel (n = 3 [23.1 %]), stomach (n = 2 [15.4 %]), rectum (n = 1 [7.7 %]), and anus (n = 1 [7.7 %]). Most patients (n = 10 [77 %]) showed isolated GI tract involvement. Eight patients had chronic inflammatory and/or immunocompromised status, including 4 with inflammatory bowel disease (all of whom underwent treatment), 3 with post-organ transplant status, and 1 with irritable bowel syndrome. All cases exhibited cytokeratin-/CD20-/PAX-5-/CD138+ and/or MUM1+ immunophenotype. Based on available data, 8 of 11 (72.7 %) patients had Epstein-Barr virus reactivation. Among 11 patients with follow-up data, the mean follow-up duration was 13.5 (range, 3-40) months; at the end of follow-up, 45.5 % of patients (5 of 11 patients) showed complete remission after chemotherapy.

Conclusion: Primary HIV-negative GI-PBL occurs predominantly in the colon of relatively elderly males with immunosuppression. Its clinical course can be heterogenous, presenting a comorbidity with inflammatory bowel disease or post-organ transplantation status.

Keywords: EBV reactivation; Gastrointestinal tract; HIV-negative; Inflammatory bowel disease; Organ transplantation; Plasmablastic lymphoma.

Publication types

Case Reports
Review

MeSH terms

Adult
Aged
Aged, 80 and over
Disease Progression
Epstein-Barr Virus Infections* / complications
Female
HIV Infections* / complications
Herpesvirus 4, Human
Humans
Inflammatory Bowel Diseases*
Male
Middle Aged
Plasmablastic Lymphoma* / diagnosis
Plasmablastic Lymphoma* / drug therapy
Plasmablastic Lymphoma* / pathology
Retrospective Studies
Stomach / pathology