Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 16, 2022; 10(17): 5708-5716
Published online Jun 16, 2022. doi: 10.12998/wjcc.v10.i17.5708
Gray zone lymphoma effectively treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab chemotherapy: A case report
Nobumasa Hojo, Makoto Nagasaki, Yasuha Mihara
Nobumasa Hojo, Department of General Medicine, National Hospital Organization Hamada Medical Center, Hamada City 697-8511, Shimane, Japan
Makoto Nagasaki, Department of Pathology, National Hospital Organization Hamada Medical Center, Hamada City 697-8511, Shimane, Japan
Yasuha Mihara, Clinical Resident, National Hospital Organization Hamada Medical Center, Hamada City 697-8511, Shimane, Japan
Author contributions: Hojo N and Mihara Y were the patient’s doctors in charge, reviewed the literature and contributed to manuscript drafting; Nagasaki M was the patient’s pathologist, made the pathological diagnosis, reviewed the literature and contributed to manuscript drafting; and All authors issued final approval for this version to be submitted.
Informed consent statement: Written informed consent was obtained from the patient for the publication of this study and any accompanying images.
Conflict-of-interest statement: The authors declare that have no conflicts of interest.
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: Nobumasa Hojo, MD, PhD, Chief Physician, Department of General Medicine, National Hospital Organization Hamada Medical Center, Asai-cho 777-12, Hamada City 697-8511, Shimane, Japan. hojo.nobumasa.qw@mail.hosp.go.jp
Received: December 11, 2021
Peer-review started: December 11, 2021
First decision: February 8, 2022
Revised: March 8, 2022
Accepted: April 3, 2022
Article in press: April 3, 2022
Published online: June 16, 2022
Processing time: 179 Days and 16 Hours
Abstract
BACKGROUND

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma (BCLu-DLBCL/cHL), also referred to as gray zone lymphoma (GZL), is known to share features with cHL and DLBCL. However, GZL is often difficult to diagnose. There is no consensus regarding the optimal therapeutic regimen. Most reported cases of GZL have been in Caucasian and Hispanic individuals, and its incidence is lower in African-American and Asian populations, including the Japanese population.

CASE SUMMARY

A 69-year-old female presented at our hospital with a growing mass on the right side of her neck. An elastic, soft mass measuring 9 cm × 6 cm was palpable in the right cervical region. Laboratory analyses showed pancytopenia, increased serum lactate dehydrogenase levels, and markedly increased levels of soluble interleukin-2 receptor. Enhanced computed tomography (CT) and fluorodeoxyglucose positron emission tomography (PET)/CT revealed multiple lesions throughout her body. She was diagnosed with GZL based on the characteristic pathological findings, the immunophenotype [CD20+, PAX5+, OCT2+/BOB1 (focal+), CD30+, CD15-], and the strong positive expression of neoplastic programmed cell death protein ligand 1 (PD-L1) in her lymphoma cells. The lymphoma was stage IV according to the Lugano classification and high-risk according to the International Prognostic Index for aggressive non-Hodgkin lymphoma. The patient received cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab (R-CHOP) chemotherapy because the tumor cells were CD20+. She has remained in complete remission for 3 years.

CONCLUSION

GZL was diagnosed based on histopathology and immunophenotyping with ancillary PD-L1 positivity. R-CHOP chemotherapy was an effective treatment.

Keywords: Classical Hodgkin lymphoma; Diffuse large B-cell lymphoma; Gray zone lymphoma; Programmed cell death protein ligand 1; R-CHOP; Case report

Core Tip: The incidence of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma (BCLu-DLBCL/cHL), also known as gray zone lymphoma (GZL), is low in Asian populations. A patient presented with right-sided cervical lymph node enlargement. The patient was diagnosed based on the characteristic pathological findings, the immunophenotype [CD20+, PAX5+, OCT2+/BOB1 (focal+), CD30+, CD15-], and the strong positive expression of neoplastic programmed cell death protein ligand 1 in her lymphoma cells. There is no consensus regarding the optimal therapeutic regimen for GZL. The addition of rituximab to the chemotherapy regimen should be considered if the tumor cells are CD20+. The patient was successfully treated with the cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab regimen and has remained in complete remission for 3 years.