A Case of POEMS Syndrome Associated with Idiopathic Liver Cirrhosis

특발성 간경변증을 동반한 POEMS 증후군 1예

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesion) is rare multisystemdisease of unknown cause with varying clinical manifestations. Although there is no established definition for this syndrome,the common classical features are considered to be secondary to the plasma cell dyscrasia with the production of a monoclonalcomponent. Anasarca, including ascites, peripheral edema, and pleural effusion, occurs frequently in POEMS syndrome, butcases with idiopathic liver cirrhosis are rarely reported. We experienced a 73-year-old female with POEMS syndrome whose mainproblem was massive ascites. It is the first report of idiopathic liver cirrhosis in a patient with POEMS syndrome in Korea.

POEMS 증후군은 다발성 신경병증(polyneuropathy), 장기종대(organomegaly), 내분비장애(endocrinopathy), 단클론성 감마글로블린병증(monoclonal gammopahty), 피부 병변(skin lesion)을 주 양상으로 발현되는 드문 증후군으로, 본 예에서는 특발성 간경변증을 동반한 POEMS 증후군 환자를 경험하였기에 보고하는 바이다.