거대세포바이러스 망막염으로 발현된 Good증후군 2예
Two Cases of Cytomegalovirus Retinitis as a Manifestation of Good's Syndrome
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Good증후군은 흉선종과 면역결핍을 나타내는 질환이다. 주로 체액성 면역결핍에 의한 세균감염이 발생하며 또한 세포성 면역결핍에 의하여 바이러스나 진균, 결핵 감염을 나타내기도 한다. Good증후군의 치료는 면역글로불린 정맥 주사이다. 시력저하를 주소로 내원한 두 환자에서 공통적으로 세포성 면역결핍에 의한 거대세포바이러스 망막염으로 발현한 Good증후군을 진단하였으며 증례 1의 환자는 폐 및 연부 조직 결핵을 동반하였고, 증례 2의 경우 거대세포바이러스 폐렴을 동반하였다. 두 환자 모두 ganciclovir 정맥주사 및 면역글로불린 치료로 호전된 바 이에 저자들은 문헌고찰과 함께 보고하는 바이다.
Abstract
Good's syndrome (GS) is a rare cause of acquired combined B- and T-cell immunodeficiency in adults. GS is also known as a rare form of paraneoplastic syndrome of thymoma that may persist after thymectomy. Some reported GS cases have been related to various diseases as a result of humoral immunodeficiency, but no report to date has related GS to cytomegalovirus (CMV) retinitis in Korea. We report two cases of CMV retinitis as a manifestation of GS with severe cellular immunodeficiency. In the first case, a 61-year-old woman was diagnosed with GS manifesting as CMV retinitis combined with coincident pulmonary tuberculosis and soft-tissue tuberculosis. In the second case, a 56-year-old man had CMV retinitis and CMV pneumonia. Both patients had a history of thymoma and had received total thymectomies, and were diagnosed with GS a few years thereafter. (Korean J Med 2011;81:266-272)
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