Lee YP, Son SM, Kwon J. Asian variant intravascular large B-cell lymphoma with highly suspected central nervous system involvement: A case report. World J Clin Cases 2023; 11(33): 8058-8064 [PMID: 38075582 DOI: 10.12998/wjcc.v11.i33.8058]
Corresponding Author of This Article
Jihyun Kwon, MD, PhD, Associate Professor, Doctor, Department of Internal Medicine, Chungbuk National University Hospital, 776, 1Sunhwan-ro, Seowon-gu, Cheongju 28644, South Korea. marioncrepe@gmail.com
Research Domain of This Article
Hematology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Nov 26, 2023; 11(33): 8058-8064 Published online Nov 26, 2023. doi: 10.12998/wjcc.v11.i33.8058
Asian variant intravascular large B-cell lymphoma with highly suspected central nervous system involvement: A case report
Yong-Pyo Lee, Seung-Myoung Son, Jihyun Kwon
Yong-Pyo Lee, Jihyun Kwon, Department of Internal Medicine, Chungbuk National University Hospital, Cheongju 28644, South Korea
Seung-Myoung Son, Department of Pathology, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju 28644, South Korea
Author contributions: Lee YP and Son SM contributed to manuscript writing and editing and to data collection; Lee YP and Kwon JH contributed to data analysis; Kwon JH contributed to conceptualization and supervision; All authors have read and approved the final manuscript.
Informed consent statement: The institutional review board of Chungbuk National University Hospital approved this study (approval number. CBNUH 2023-04-024), and informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors declare that they have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jihyun Kwon, MD, PhD, Associate Professor, Doctor, Department of Internal Medicine, Chungbuk National University Hospital, 776, 1Sunhwan-ro, Seowon-gu, Cheongju 28644, South Korea. marioncrepe@gmail.com
Received: September 3, 2023 Peer-review started: September 3, 2023 First decision: October 24, 2023 Revised: November 1, 2023 Accepted: November 10, 2023 Article in press: November 10, 2023 Published online: November 26, 2023 Processing time: 81 Days and 21.6 Hours
Abstract
BACKGROUND
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal lymphoma. In particular, the Asian variant of IVLBCL is characterized by hemophagocytic lymphohistiocytosis along with bone marrow involvement. However, central nervous system (CNS) involvement is uncommon in this variant compared to the Western variant. Here, we report a case of typical Asian variant IVLBCL with highly suspected CNS involvement and discuss the nature of the disease and its genetic aberration.
CASE SUMMARY
A 67-year-old female patient complained of gradually worsening cognitive impairment. While hospitalized, she developed a high fever and showed marked bicytopenia. Intracranial imaging revealed a suspected leptomeningeal disease. Although no malignant cells were found in the cerebrospinal fluid (CSF), the protein and lactate dehydrogenase levels in CSF were increased. Bone marrow examination revealed an increased number of hemophagocytic histiocytes, and 18F-fluorodeoxyglucose (FDG) positron emission tomography with computerized tomography scan revealed increased FDG uptake in both adrenal glands, the liver, and the right ethmoid sinus. A tissue biopsy showed atypical large lymphoid cells with prominent nucleoli in the vessels, and the tumor cells were positive for CD20, BCL2, BCL6, and IRF4/MUM1. In addition, targeted sequencing identified MYD88, TET2, and PIM1 mutations. Consequently, we diagnosed the patient with the Asian variant of IVLBCL with highly suspected CNS involvement.
CONCLUSION
Suspicion of IVLBCL and immediate diagnosis lead to timely treatment. Moreover, careful CNS examination at diagnosis is recommended.
Core Tip: Intravascular large B-cell lymphoma (IVLBCL) is a rare but clinically aggressive lymphoproliferative disease. Given its aggressive nature, immediate diagnosis of IVLBCL and timely treatment are critical for better clinical outcomes. As central nervous system (CNS) involvement adversely affects prognosis if IVLBCL, active CNS examination is required at diagnosis. In addition, along with conventional pathology, targeted sequencing contributes to diagnosis and provides a basis for use of targeted agents. Here, we report a case of Asian variant IVLBCL with highly suspected CNS involvement. We first describe the clinical course of disease and then discuss the genetic aberrations found in the patient.
