Sang Hyun Kim; Han Ah Lee; Seung Woon Park; Kang Won Lee; Young Ho Seo; Jae Hoon Kim; Sung Jae Choi; Young Ho Lee; Jong Dae Ji; Gwan Gyu Song

doi:10.4078/jrd.2016.23.1.61

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Kim, Lee, Park, Lee, Seo, Kim, Choi, Lee, Ji, and Song: A Case of Non-Hodgkin's Lymphoma of the Ocular Adnexa and Lung in a 17-year-old with Primary Sjögren's Syndrome

Case Report

J Rheum Dis 2016;23(1):61-65.

Published online: 31 October 2016

DOI: https://doi.org/10.4078/jrd.2016.23.1.61

A Case of Non-Hodgkin's Lymphoma of the Ocular Adnexa and Lung in a 17-year-old with Primary Sjögren's Syndrome

Sang Hyun Kim, Han Ah Lee, Seung Woon Park, Kang Won Lee, Young Ho Seo, Jae Hoon Kim, Sung Jae Choi, Young Ho Lee, Jong Dae Ji, Gwan Gyu Song

Division of Rheumatology, Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea

Corresponding to: Sung Jae Choi, Division of Rheumatology, Department of Internal Medicine, Korea University Ansan Hospital, 123 Jeokgeum-ro, Danwon-gu, Ansan 15355, Korea. E-mail: csjmd@hotmail.com

Received 6 March 2015 Revised 16 April 2015 Accepted 24 April 2015

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocyte-mediated destruction of exocrine glands, which produces classical symptoms of dry eyes and dry mouth. Aside from the clinical manifestations associated with exocrine glands, extraglandular features of SS include a major long-term concern for development of lymphoma. The lifetime risk of non-Hodgkin's lymphoma (NHL) in an SS patient is approximately 5% to 10%, 20 times higher than that of the normal population. This case report describes a rare occurrence of NHL in the eyelid and lung of an adolescent female with SS, whose disease activity had been monitored closely. This is the first reported case in Korea.

Keywords: Sjögren's syndrome, Lymphoma, Conjunctiva, Lung

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Figure 1.

Orbital computed tomography scan (A, B) showed no abnormal findings in the or-bits, eyeballs, extraocular muscles, or optic nerves.

Figure 2.

Conjunctival lymphoid tissue. H&E stained sctions showing abnormal lymphoid architecture and atypical lymphoid cells with mitosis (A: ×40, B: ×400).

Figure 3.

Immunohistochemistry showing extensive, dense, packed CD20 B-cell lymphoid infiltrate (immunohistochemistry with hematoxylin counterstain, ×400).

Figure 4.

Multifocal patchy consolidations and ground glass opacities in the right middle lobe (arrow).

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