일차성 쇼그렌 증후군 환자에서 발생한 눈물샘의 점막연관 림프조직형 림프종 1예
A Case of Lacrimal Gland MALT Lymphoma in a Patient with Primary Sjögren’s Syndrome
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목적: 일차성 쇼그렌 증후군과 베체트병을 동시에 진단 받은 환자에서 발생한 눈물샘의 점막연관 림프조직형 림프종을 항암화학요법을 통해 효과적으로 치료하였기에 이를 보고하고자 한다. 증례요약: 8년 전 쇼그렌 증후군과 베체트병을 진단 받은 49세 여자 환자가 1년 전부터 발생한 우안 위, 아래 눈꺼풀의 무통성 부종을 주소로 내원하였다. 조직 검사 결과 악성 점막연관 림프조직형 림프종으로 진단되었고 전신 전이나 골수 침범은 없어 항암화학요법을 시행하였다. 8차 항암요법 이후 양안 눈꺼풀은 외형적으로 정상, 영상학적으로 부분 관해 소견을 보여 경과관찰 중이다. 결론: 쇼그렌 증후군 등의 자가면역 질환이 있는 환자에서 눈꺼풀 부종이나 종괴가 촉지되는 경우에는 점막연관 림프조직형 림프종의 합병 가능성을 염두에 두어야 한다.
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.
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