A Case of POEMS Syndrome Associated with Idiopathic Liver Cirrhosis / 대한내과학회지

ABSTRACT

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesion) is rare multisystem disease 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 monoclonal component. Anasarca, including ascites, peripheral edema, and pleural effusion, occurs frequently in POEMS syndrome, but cases with idiopathic liver cirrhosis are rarely reported. We experienced a 73-year-old female with POEMS syndrome whose main problem was massive ascites. It is the first report of idiopathic liver cirrhosis in a patient with POEMS syndrome in Korea.