Preliminary Exploration Of The Pathogenesy Of POEMS Syndrome (Clinical Analysis Of Five Cases)

Objective In order to describe the clinical features of POEMS syndrome, comprehend its pathogenesy and elevate the cognition of the syndrome. Methods Five cases of POEMS syndrome in our hospital from the year 1994 to 2005 were reported. The clinical features, laboratory inspections , relevant therapeutics and the outcome of five cases were analyzed and the literatures were reviewed.Results All five cases had peripheral polyneuropathy, endocrinopathy, organomegaly, skin change and edema, but no case had M-protein in serum protein electrophoresis. Five cases were treated with glucocorticoid or glucocorticoid combined with immunodepressant and three patients' symptoms were improved subsequently.Conclusions POEMS syndrome is a rare multisystem disorder with some symptom complexes including polyneuropathy, organomegaly,endocrinopathy, M-proteins , skin changes and edema. The pathogenesy may associate with infection of human herpesvirus-8(HHV-8), overexpression of vascular endothelial growth factor(VEGF) and proinflammatory cytokines like interleukin-6, interleukin-1β and tumor necrosis factor-α (TNF-α ), paraplasm of plasma cells and so on. It is easily misdiagnosed in early stage. More attention should be paid to it for improving the accuracy of diagnosis and adequacy of the treatment. The detection rate of M-protein using serum protein electrophoresis is low, so in china, M-protein seems to be nonessential for the diagnosis. Using immunofixation electrophoresis can elevate the detection rate of M-protein and is helpful to the diagnosis.