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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| Format: | Article |
| Language: | English |
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MDPI AG
2020-12-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/9/12/3997 |
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| author | Giuseppe Ingravallo Eugenio Maiorano Marco Moschetta Luisa Limongelli Mauro Giuseppe Mastropasqua Gisella Franca Agazzino Vincenzo De Ruvo Paola Tarantino Gianfranco Favia Saverio Capodiferro |
| author_facet | Giuseppe Ingravallo Eugenio Maiorano Marco Moschetta Luisa Limongelli Mauro Giuseppe Mastropasqua Gisella Franca Agazzino Vincenzo De Ruvo Paola Tarantino Gianfranco Favia Saverio Capodiferro |
| author_sort | Giuseppe Ingravallo |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-10T14:11:26Z |
| format | Article |
| id | doaj.art-27adef233a0a4cc6939b6710a7ce9125 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-10T14:11:26Z |
| publishDate | 2020-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-27adef233a0a4cc6939b6710a7ce91252023-11-21T00:07:30ZengMDPI AGJournal of Clinical Medicine2077-03832020-12-01912399710.3390/jcm9123997Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant LiteratureGiuseppe Ingravallo0Eugenio Maiorano1Marco Moschetta2Luisa Limongelli3Mauro Giuseppe Mastropasqua4Gisella Franca Agazzino5Vincenzo De Ruvo6Paola Tarantino7Gianfranco Favia8Saverio Capodiferro9Department of Emergency and Organ Transplantation—Section of Pathology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Section of Pathology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Breast Unit, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Interdisciplinary Medicine—Section of Odontostomatology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Section of Pathology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Section of Pathology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Breast Unit, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Emergency and Organ Transplantation—Section of Pathology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Interdisciplinary Medicine—Section of Odontostomatology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyDepartment of Interdisciplinary Medicine—Section of Odontostomatology, University of Bari Aldo Moro, Piazza G. Cesare, 11, 70124 Bari, ItalyThe 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.https://www.mdpi.com/2077-0383/9/12/3997autoimmune diseasesSjögren syndromeminor salivary glandsB-cell lymphomaextranodal marginal zone lymphomaMALT lymphoma |
| spellingShingle | Giuseppe Ingravallo Eugenio Maiorano Marco Moschetta Luisa Limongelli Mauro Giuseppe Mastropasqua Gisella Franca Agazzino Vincenzo De Ruvo Paola Tarantino Gianfranco Favia Saverio Capodiferro Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature Journal of Clinical Medicine autoimmune diseases Sjögren syndrome minor salivary glands B-cell lymphoma extranodal marginal zone lymphoma MALT lymphoma |
| 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 sjogren syndrome case presentation and relevant literature |
| topic | autoimmune diseases Sjögren syndrome minor salivary glands B-cell lymphoma extranodal marginal zone lymphoma MALT lymphoma |
| url | https://www.mdpi.com/2077-0383/9/12/3997 |
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