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Lacrimal gland lymphoma in Sjögren syndrome

Poster Details

First Author: M.Palamar TURKEY

Co Author(s):    F. Sahin   N. Ozsan                 

Abstract Details

Purpose:

To report a bilateral lacrimal gland lymphoma in a female with Primary Sjögren Syndrome (pSS).

Setting:

Ege University Faculty of Medicine Departments of Ophthalmology, Haematology, Pathology

Methods:

A 31 year-old female with pSS experiencing bilateral lacrimal gland puffiness for 3 years was evaluated.

Results:

Both lacrimal glands were hypertrophic and edematous. Schirmer 1 score was 2 and 1 mm, 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 was performed. Immunohistopathologic evaluation revealed diffuse and intense CD20, CD5 and bcl-2 positivity with negative cyclin D1 and CD23. The results supported lymphoma however, the exact histological discrimination could not be made. The patient was sent to haematology department and as lacrimal gland was affected extranodal marginal zone lymphoma was diagnosed. CHOP treatment was initiated.

Conclusions:

Primary Sjögren Syndrome is an autoimmune disease associated with an increased risk of lymphoma. Lymphomas complicating pSS are mostly low-grade B cell non-Hodgkin lymphomas, predominantly of marginal zone histological type. Early detection with histopathologic comfirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients.

Financial Disclosure:

NONE

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