다량의 심낭 삼출로 발현된 POEMS 증후군 1예
A Case of POEMS Syndrome that Presented with a Moderate Pericardial Effusion
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초록
심낭 삼출은 악성종양, 결핵 등 다양한 원인에 의해 유발되는 질환으로 과거와는 달리 선험적인 치료에 의존하기 보다 정확한 원인 규명의 중요성이 점차 커지고 있다. 저자들은 심낭 삼출을 주된 증상으로 하면서 말초신경병증, 비장 비대, 갑상샘저하증, 단세포감마글로불린병증, 피부색소침착, 다모증, 흉막 삼출, 전신 부종, 폐동맥고혈압 등을 동반한 POEMS 증후군 1예를 경험하여 문헌고찰과 함께 보고한다. 심낭 삼출의 치료에 반응이 없으면서 원인을 찾지 못하여 특발성 심낭염으로 진단한 환자의 추가적인 감별 진단의 하나로 POEMS 증후군이 고려되어야 하겠다.
Abstract
Pericardial effusions can be caused by a wide variety of infectious or noninfectious diseases. After a conventional diagnostic work-up, the etiology of pericardial effusion often remains idiopathic. We report a patient with POEMS syndrome whose main clinical problem was recurrent pericardial effusions. Patients with POEMS syndrome often have generalized edema and a pleural effusion, while a pericardial effusion is a very rare complication. A 44-year-old man visited our hospital because of a recurrent pericardial effusion. He was initially diagnosed with idiopathic pericarditis five months prior. We reassessed the patient meticulously and found IgG lambda type monoclonal gammopathy, polyneuropathy, lymphadenopathy, peripheral edema, pleural effusion, hypothyroidism, pulmonary hypertension, hyperpigmentation, hypertrichosis, and papilledema, which we diagnosed as POEMS syndrome. (Korean J Med 2011;81:245-250)
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