Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses

A rare subtype of diffuse large B-cell lymphoma (DLBCL) has been reported to be accompanied by elevated immunoglobulin M (IgM) paraprotein in the serum at diagnosis, called as IgMs-DLBCL. The monoclonal IgM paraprotein disappears soon after treatment in most of these patients. Here, we described a D...

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Main Authors: Fei Xiao, Yong-Mei Cai, Jian-Chen Fang, Yan-Ying Shen, Bao-Hua Yu, Yi-Wei Zhang, Di Zhu, Zi-Hua Li, Guo-Qing Li, Jian Hou, Min-Yue Zhang, Hong-Hui Huang
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-11-01
Series:Frontiers in Genetics
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Online Access:https://www.frontiersin.org/articles/10.3389/fgene.2023.1228372/full
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author Fei Xiao
Yong-Mei Cai
Jian-Chen Fang
Yan-Ying Shen
Bao-Hua Yu
Yi-Wei Zhang
Di Zhu
Zi-Hua Li
Guo-Qing Li
Jian Hou
Min-Yue Zhang
Hong-Hui Huang
author_facet Fei Xiao
Yong-Mei Cai
Jian-Chen Fang
Yan-Ying Shen
Bao-Hua Yu
Yi-Wei Zhang
Di Zhu
Zi-Hua Li
Guo-Qing Li
Jian Hou
Min-Yue Zhang
Hong-Hui Huang
author_sort Fei Xiao
collection DOAJ
description A rare subtype of diffuse large B-cell lymphoma (DLBCL) has been reported to be accompanied by elevated immunoglobulin M (IgM) paraprotein in the serum at diagnosis, called as IgMs-DLBCL. The monoclonal IgM paraprotein disappears soon after treatment in most of these patients. Here, we described a DLBCL patient with continuously elevated IgM following therapy. A 59-year-old male was diagnosed with DLBCL (GCB subtype per Hans algorithm, stage IA) with involvement of the right cervical lymph node. After six cycles of immuno-chemotherapy with the R-CHOP regimen, complete metabolic remission was achieved, but an elevated level of serum IgM persisted. To investigate the origin of elevated IgM, pathologic, immunophenotypic, and molecular analyses of lymph node and bone marrow (BM) samples were performed pre- and post-treatment. BM infiltration of lymphoplasmacytic cells, and a typical immunophenotypic profile by flow cytometry supported the diagnosis of Waldenström macroglobulinemia (WM). The MCD subtype of DLBCL was identified by next-generation sequencing of the lymph node at initial diagnosis characterized by co-occurring point mutations in MYD88L265P and CD79B. Additionally, two different dominant clonotypes of the immunoglobulin heavy chain (IGH) were detected in the lymph node and BM by IGH sequencing, which was IGHV 3–11*06/IGHJ 3*02 and IGHV 3–11*06/IGHJ 6*02, respectively, speculating to be two independent clonal origins. This study will provide a panoramic understanding of the origin or biological characteristics of DLBCL co-occurring with WM.
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spelling doaj.art-105c5567f20746f69424b6ee40b1bd642023-11-06T15:52:44ZengFrontiers Media S.A.Frontiers in Genetics1664-80212023-11-011410.3389/fgene.2023.12283721228372Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analysesFei Xiao0Yong-Mei Cai1Jian-Chen Fang2Yan-Ying Shen3Bao-Hua Yu4Yi-Wei Zhang5Di Zhu6Zi-Hua Li7Guo-Qing Li8Jian Hou9Min-Yue Zhang10Hong-Hui Huang11Department of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Pathology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Pathology, Fudan University Shanghai Cancer Center, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaShanghai Rightongene Biomedical Technology Co., Ltd., Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaDepartment of Hematology, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, ChinaA rare subtype of diffuse large B-cell lymphoma (DLBCL) has been reported to be accompanied by elevated immunoglobulin M (IgM) paraprotein in the serum at diagnosis, called as IgMs-DLBCL. The monoclonal IgM paraprotein disappears soon after treatment in most of these patients. Here, we described a DLBCL patient with continuously elevated IgM following therapy. A 59-year-old male was diagnosed with DLBCL (GCB subtype per Hans algorithm, stage IA) with involvement of the right cervical lymph node. After six cycles of immuno-chemotherapy with the R-CHOP regimen, complete metabolic remission was achieved, but an elevated level of serum IgM persisted. To investigate the origin of elevated IgM, pathologic, immunophenotypic, and molecular analyses of lymph node and bone marrow (BM) samples were performed pre- and post-treatment. BM infiltration of lymphoplasmacytic cells, and a typical immunophenotypic profile by flow cytometry supported the diagnosis of Waldenström macroglobulinemia (WM). The MCD subtype of DLBCL was identified by next-generation sequencing of the lymph node at initial diagnosis characterized by co-occurring point mutations in MYD88L265P and CD79B. Additionally, two different dominant clonotypes of the immunoglobulin heavy chain (IGH) were detected in the lymph node and BM by IGH sequencing, which was IGHV 3–11*06/IGHJ 3*02 and IGHV 3–11*06/IGHJ 6*02, respectively, speculating to be two independent clonal origins. This study will provide a panoramic understanding of the origin or biological characteristics of DLBCL co-occurring with WM.https://www.frontiersin.org/articles/10.3389/fgene.2023.1228372/fulldiffuse large B-cell lymphomaWaldenström macroglobulinemiaimmunoglobulin Mimmunoglobulin heavy-chain sequencingMYD88 mutation
spellingShingle Fei Xiao
Yong-Mei Cai
Jian-Chen Fang
Yan-Ying Shen
Bao-Hua Yu
Yi-Wei Zhang
Di Zhu
Zi-Hua Li
Guo-Qing Li
Jian Hou
Min-Yue Zhang
Hong-Hui Huang
Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
Frontiers in Genetics
diffuse large B-cell lymphoma
Waldenström macroglobulinemia
immunoglobulin M
immunoglobulin heavy-chain sequencing
MYD88 mutation
title Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
title_full Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
title_fullStr Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
title_full_unstemmed Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
title_short Diffuse large B-cell lymphoma with continuously elevated immunoglobulin M following treatment: a case report with pathologic, immunophenotypic, and molecular analyses
title_sort diffuse large b cell lymphoma with continuously elevated immunoglobulin m following treatment a case report with pathologic immunophenotypic and molecular analyses
topic diffuse large B-cell lymphoma
Waldenström macroglobulinemia
immunoglobulin M
immunoglobulin heavy-chain sequencing
MYD88 mutation
url https://www.frontiersin.org/articles/10.3389/fgene.2023.1228372/full
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