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Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stim...

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Autores principales: Ingravallo, Giuseppe, Maiorano, Eugenio, Moschetta, Marco, Limongelli, Luisa, Mastropasqua, Mauro Giuseppe, Agazzino, Gisella Franca, De Ruvo, Vincenzo, Tarantino, Paola, Favia, Gianfranco, Capodiferro, Saverio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7764276/
https://www.ncbi.nlm.nih.gov/pubmed/33321727
http://dx.doi.org/10.3390/jcm9123997
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author Ingravallo, Giuseppe
Maiorano, Eugenio
Moschetta, Marco
Limongelli, Luisa
Mastropasqua, Mauro Giuseppe
Agazzino, Gisella Franca
De Ruvo, Vincenzo
Tarantino, Paola
Favia, Gianfranco
Capodiferro, Saverio
author_facet Ingravallo, Giuseppe
Maiorano, Eugenio
Moschetta, Marco
Limongelli, Luisa
Mastropasqua, Mauro Giuseppe
Agazzino, Gisella Franca
De Ruvo, Vincenzo
Tarantino, Paola
Favia, Gianfranco
Capodiferro, Saverio
author_sort Ingravallo, Giuseppe
collection PubMed
description The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patients.
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spelling pubmed-77642762020-12-27 Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature Ingravallo, Giuseppe Maiorano, Eugenio Moschetta, Marco Limongelli, Luisa Mastropasqua, Mauro Giuseppe Agazzino, Gisella Franca De Ruvo, Vincenzo Tarantino, Paola Favia, Gianfranco Capodiferro, Saverio J Clin Med Article The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patients. MDPI 2020-12-10 /pmc/articles/PMC7764276/ /pubmed/33321727 http://dx.doi.org/10.3390/jcm9123997 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Ingravallo, Giuseppe
Maiorano, Eugenio
Moschetta, Marco
Limongelli, Luisa
Mastropasqua, Mauro Giuseppe
Agazzino, Gisella Franca
De Ruvo, Vincenzo
Tarantino, Paola
Favia, Gianfranco
Capodiferro, Saverio
Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title_full Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title_fullStr Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title_full_unstemmed Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title_short Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature
title_sort primary breast extranodal marginal zone lymphoma in primary sjögren syndrome: case presentation and relevant literature
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7764276/
https://www.ncbi.nlm.nih.gov/pubmed/33321727
http://dx.doi.org/10.3390/jcm9123997
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