POEMS syndrome

Abstract

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym refers to several, but not all, of the features of the syndrome: Polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Other important features not included in the POEMS acronym include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, a predisposition towards thrombosis, and abnormal pulmonary function tests. The prevalence is estimated to be 0.3 per 100,000. The pathogenesis of the syndrome is not understood but VEGF is the cytokine that correlates best with disease activity. In more than 95% of cases, the plasma cells are lambda light chain restricted. Treatment focuses on treating the underlying clone and supportive care. All effective therapies have been borrowed from the multiple myeloma armamentarium. The majority of patients respond to therapy, and patients’ survival is superior to that of patients with multiple myeloma.