Poems syndrome

A 40 year old Chinese woman presented with anasarca and later developed features of POEMS syndrome. These are peripheral neuropathy (P), organomegaly (O), endocrine dysfunction (E), monoclonal gammopathy (M), and skin changes (S) which are usually associated with plasma cell dyscrasia. In our patient, monoclonal gammopathy was not detected on immunofixation electrophoresis and was revealed only after analysis of kappa/lambda light chain ratio of the raised serum IgA immunoglobulin. Needle liver biopsy of her grossly enlarged liver showed marked accumulation of glycogen and presence of giant mitochondria in the hepatocytes, a feature not previously reported.