Hyo Won Kim; Yoon Mi Sung; Ji Sun Baik; Suk-Woo Yang

doi:10.3341/jkos.2016.57.7.1159

Journal List > J Korean Ophthalmol Soc > v.57(7) > 1010349

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Kim, Sung, Baik, and Yang: A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome

Case Report

J Korean Ophthalmol Soc 2016;57(7):1159-1164.

Published online: 25 September 2016

DOI: https://doi.org/10.3341/jkos.2016.57.7.1159

A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren's Syndrome

Hyo Won Kim, MD, Yoon Mi Sung, MD, Ji Sun Baik, MD, Suk-Woo Yang, MD, PhD

Department of Ophthalmology and Visual Science, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea

Address reprint requests to Suk-Woo Yang, MD, PhD Department of Ophthalmology, The Catholic University of Korea Seoul St. Mary's Hospital, #222 Banpo-daero, Seocho-gu, Seoul 06591, Korea Tel: 82-2-2258-1200, Fax: 82-2-599-7405 E-mail: yswoph@hanmail.net

Received 8 October 2015 Accepted 5 February 2016

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

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.

Keywords: Autoimmune disease, Behcet's disease, Lymphoma of lacrimal gland, Mucosa-associated lymphoid tissue (MALT) lymphoma, Sjögren's syndrome

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Figure 1.

External photograph of a 49-year-old female patient with lacrimal gland mucosa-associated lymphoid tissue lymphoma. (A) Before chemotherapy, swelling of upper and lower eyelids was prominent in the right eye. (B) One month after eight cycles of chemotherapy, the right eyelid appears nearly normal.

Figure 2.

Orbital T1-weighted magnetic resonance images showing enhancing enlargement of the right lacrimal gland. (A, B) Before chemotherapy, a diffusely enlarged right lacrimal gland is noted. (C, D) One month after eight cycles of chemotherapy, the right lacrimal gland enhancement reduced dramatically.

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