Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome

A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, re...

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Detalles Bibliográficos
Autores principales: Palamar, Melis, Ozsan, Nazan, Sahin, Fahri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5050356/
https://www.ncbi.nlm.nih.gov/pubmed/27738539
http://dx.doi.org/10.1155/2016/2798304
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author Palamar, Melis
Ozsan, Nazan
Sahin, Fahri
author_facet Palamar, Melis
Ozsan, Nazan
Sahin, Fahri
author_sort Palamar, Melis
collection PubMed
description A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone) treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients.
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spelling pubmed-50503562016-10-13 Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome Palamar, Melis Ozsan, Nazan Sahin, Fahri Case Rep Ophthalmol Med Case Report A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone) treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients. Hindawi Publishing Corporation 2016 2016-09-21 /pmc/articles/PMC5050356/ /pubmed/27738539 http://dx.doi.org/10.1155/2016/2798304 Text en Copyright © 2016 Melis Palamar et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Palamar, Melis
Ozsan, Nazan
Sahin, Fahri
Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title_full Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title_fullStr Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title_full_unstemmed Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title_short Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome
title_sort bilateral lacrimal gland lymphoma in sjögren syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5050356/
https://www.ncbi.nlm.nih.gov/pubmed/27738539
http://dx.doi.org/10.1155/2016/2798304
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AT ozsannazan bilaterallacrimalglandlymphomainsjogrensyndrome
AT sahinfahri bilaterallacrimalglandlymphomainsjogrensyndrome

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