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Case Reports in Ophthalmological Medicine
Volume 2016, Article ID 2798304, 3 pages
Case Report

Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome

1Department of Ophthalmology, Faculty of Medicine, Ege University, Izmir, Turkey
2Department of Pathology, Faculty of Medicine, Ege University, Izmir, Turkey
3Department of Hematology, Faculty of Medicine, Ege University, Izmir, Turkey

Received 14 July 2016; Accepted 28 August 2016

Academic Editor: Alexander A. Bialasiewicz

Copyright © 2016 Melis Palamar et al. 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.


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.