J Korean Ophthalmol Soc > Volume 57(7); 2016 > Article
Journal of the Korean Ophthalmological Society 2016;57(7):1159-1164.
DOI: https://doi.org/10.3341/jkos.2016.57.7.1159    Published online July 15, 2016.
A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome.
Hyo Won Kim, Yoon Mi Sung, Ji Sun Baik, Suk Woo Yang
Department of Ophthalmology and Visual Science, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea. yswoph@hanmail.net
일차성 쇼그렌 증후군 환자에서 발생한 눈물샘의 점막연관 림프조직형 림프종 1예
가톨릭대학교 의과대학 서울성모병원 안과 및 시과학교실
To report a case of lacrimal gland mucosa-associated lymphoid tissue (MALT) lymphoma in a patient with primary Sjögren's syndrome and Behcet's disease. CASE SUMMARY: A 49-year-old female patient with primary Sjögren's syndrome and Behcet's disease presented with a one-year history of painless upper and lower eyelid swelling in her right eye. Lacrimal gland incisional biopsy was performed, and the patient was diagnosed with malignant lymphoma (extranodal marginal zone B cell lymphoma of MALT). No distant metastases were detected on whole-body computed tomography or positron emission tomography, and the patient was treated with Rituximab, Cyclophosphamide, Vincristine, Prednisone (R-CVP) regimen chemotherapy. After 8 consecutive chemotherapy cycles, her eyelids appeared normal externally, and partial regression was found radiologically. CONCLUSIONS: The possibility of MALT lymphoma should be considered as a differential diagnosis if patients with autoimmune diseases such as primary Sjögren's syndrome show eyelid swelling or palpable mass.
Key Words: Autoimmune disease;Behcet's disease;Lymphoma of lacrimal gland;Mucosa-associated lymphoid tissue (MALT) lymphoma;Sjögren's syndrome

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