Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report

Abstract Background Epstein-Barr virus is associated with many human hematopoietic neoplasms; however, Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma is extremely rare. In routine clinical practice, detection of mucosa-associated lymphoid tissue lymphoma and diffuse large B-c...

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Main Authors: Vadim R. Gorodetskiy, Natalya A. Probatova, Dmitry M. Konovalov, Natalya V. Ryzhikova, Yulia V. Sidorova, Andrey B. Sudarikov, Olga V. Mukhortova
Format: Article
Language:English
Published: BMC 2020-01-01
Series:Journal of Medical Case Reports
Subjects:
Online Access:https://doi.org/10.1186/s13256-019-2331-1
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author Vadim R. Gorodetskiy
Natalya A. Probatova
Dmitry M. Konovalov
Natalya V. Ryzhikova
Yulia V. Sidorova
Andrey B. Sudarikov
Olga V. Mukhortova
author_facet Vadim R. Gorodetskiy
Natalya A. Probatova
Dmitry M. Konovalov
Natalya V. Ryzhikova
Yulia V. Sidorova
Andrey B. Sudarikov
Olga V. Mukhortova
author_sort Vadim R. Gorodetskiy
collection DOAJ
description Abstract Background Epstein-Barr virus is associated with many human hematopoietic neoplasms; however, Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma is extremely rare. In routine clinical practice, detection of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in a tissue sample presumes a clonal relation between these neoplasms and that diffuse large B-cell lymphoma developed by transformation of the mucosa-associated lymphoid tissue lymphoma. However, evidence to support this presumption is sparse and controversial. Assessment of the clonal relationship of the lymphoid components of a composite lymphoma is important for understanding its pathogenesis and correct diagnosis. Case presentation We present an unusual case of composite lymphoma (Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma/Epstein-Barr virus-negative diffuse large B-cell lymphoma) in the parotid salivary gland of a 62-year-old Caucasian woman with Sjögren’s syndrome and rheumatoid arthritis. Simultaneous occurrence of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in the parotid salivary gland led us to initially assume a clonal relationship between diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. Epstein-Barr virus was detected by in situ hybridization and polymerase chain reaction in the mucosa-associated lymphoid tissue lymphoma, but not in diffuse large B-cell lymphoma, suggesting that these lymphomas were not clonally related. Fragment analysis of frame region 3 polymerase chain reaction products from microdissected mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma components revealed different clonal pattern rearrangements of the immunoglobulin heavy chain gene. Conclusions Our patient’s case highlights the importance of assessing the clonal relationships of the lymphoid components of a composite lymphoma and Epstein-Barr virus screening in mucosa-associated lymphoid tissue lymphoma in patients with autoimmune disease.
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spelling doaj.art-1a606985664a49cb937370bf0cd345292022-12-21T21:27:32ZengBMCJournal of Medical Case Reports1752-19472020-01-011411710.1186/s13256-019-2331-1Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case reportVadim R. Gorodetskiy0Natalya A. Probatova1Dmitry M. Konovalov2Natalya V. Ryzhikova3Yulia V. Sidorova4Andrey B. Sudarikov5Olga V. Mukhortova6Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of RheumatologyDepartment of Pathology, N.N. Blokhin Russian Cancer Research CenterDepartment of Pathology, Dmitry Rogachev Research and Clinical Centre of Pediatric Hematology, Oncology and ImmunologyLaboratory of Molecular Hematology, National Hematology Research CenterLaboratory of Molecular Hematology, National Hematology Research CenterLaboratory of Molecular Hematology, National Hematology Research CenterDepartment of Nuclear Diagnostics, A.N. Bakulev National Medical Research Center of Cardiovascular SurgeryAbstract Background Epstein-Barr virus is associated with many human hematopoietic neoplasms; however, Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma is extremely rare. In routine clinical practice, detection of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in a tissue sample presumes a clonal relation between these neoplasms and that diffuse large B-cell lymphoma developed by transformation of the mucosa-associated lymphoid tissue lymphoma. However, evidence to support this presumption is sparse and controversial. Assessment of the clonal relationship of the lymphoid components of a composite lymphoma is important for understanding its pathogenesis and correct diagnosis. Case presentation We present an unusual case of composite lymphoma (Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma/Epstein-Barr virus-negative diffuse large B-cell lymphoma) in the parotid salivary gland of a 62-year-old Caucasian woman with Sjögren’s syndrome and rheumatoid arthritis. Simultaneous occurrence of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in the parotid salivary gland led us to initially assume a clonal relationship between diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. Epstein-Barr virus was detected by in situ hybridization and polymerase chain reaction in the mucosa-associated lymphoid tissue lymphoma, but not in diffuse large B-cell lymphoma, suggesting that these lymphomas were not clonally related. Fragment analysis of frame region 3 polymerase chain reaction products from microdissected mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma components revealed different clonal pattern rearrangements of the immunoglobulin heavy chain gene. Conclusions Our patient’s case highlights the importance of assessing the clonal relationships of the lymphoid components of a composite lymphoma and Epstein-Barr virus screening in mucosa-associated lymphoid tissue lymphoma in patients with autoimmune disease.https://doi.org/10.1186/s13256-019-2331-1Composite lymphomaMucosa-associated lymphoid tissue lymphomaDiffuse large B-cell lymphomaEpstein-Barr virusAutoimmune diseases
spellingShingle Vadim R. Gorodetskiy
Natalya A. Probatova
Dmitry M. Konovalov
Natalya V. Ryzhikova
Yulia V. Sidorova
Andrey B. Sudarikov
Olga V. Mukhortova
Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
Journal of Medical Case Reports
Composite lymphoma
Mucosa-associated lymphoid tissue lymphoma
Diffuse large B-cell lymphoma
Epstein-Barr virus
Autoimmune diseases
title Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
title_full Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
title_fullStr Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
title_full_unstemmed Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
title_short Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report
title_sort composite epstein barr virus positive mucosa associated lymphoid tissue lymphoma and epstein barr virus negative diffuse large b cell lymphoma in the parotid salivary gland of a patient with sjogren s syndrome and rheumatoid arthritis a case report
topic Composite lymphoma
Mucosa-associated lymphoid tissue lymphoma
Diffuse large B-cell lymphoma
Epstein-Barr virus
Autoimmune diseases
url https://doi.org/10.1186/s13256-019-2331-1
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