Gray Zone Lymphoma Effectively Treated with Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone, and Rituximab Chemotherapy: A Case Report

Overview

Journal World J Clin Cases

Publisher Baishideng Publishing Group

Specialty General Medicine

Date 2022 Aug 18

PMID 35979119

Authors

Nobumasa Hojo

Makoto Nagasaki

Yasuha Mihara

Affiliations

Soon will be listed here.

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.

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