Marina Scarlato1, Stefano C Previtali. 1. Division of Neuroscience, Department of Neurology & INSPE, San Raffaele Scientific Institute, Milan, Italy. scarlato.marina@hsr.it
Abstract
PURPOSE OF REVIEW: The aim is to provide an up-to-date overview of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome with special regard to the available therapy options. RECENT FINDINGS: In the past 20 years this rare plasmaproliferative disorder has been extensively characterized from a clinical point of view with complete description of the typical features as well as of other organ involvement not considered in the acronym as nephropathy or pachimeningitis. In this syndrome, the serum levels of vascular endothelial growth factor (VEGF) are abnormally elevated and now this is considered one of the major criteria for making the diagnosis. VEGF has also a prognostic value, as it decreases in response to therapy and definitely has a pathogenetic role in the multisystem involvement of POEMS. Recently great advance occurred in the treatment of POEMS syndrome with new immunomodulatory drugs such as lenalidomide, autologous peripheral blood stem cell transplantation or bevacizumab, an anti-VEGF monoclonal antibody. SUMMARY: Although many aspects of POEMS syndrome remain unclear, a valid biomarker of disease, VEGF, is available for diagnosis as well as a wide range of therapeutic options.
PURPOSE OF REVIEW: The aim is to provide an up-to-date overview of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome with special regard to the available therapy options. RECENT FINDINGS: In the past 20 years this rare plasmaproliferative disorder has been extensively characterized from a clinical point of view with complete description of the typical features as well as of other organ involvement not considered in the acronym as nephropathy or pachimeningitis. In this syndrome, the serum levels of vascular endothelial growth factor (VEGF) are abnormally elevated and now this is considered one of the major criteria for making the diagnosis. VEGF has also a prognostic value, as it decreases in response to therapy and definitely has a pathogenetic role in the multisystem involvement of POEMS. Recently great advance occurred in the treatment of POEMS syndrome with new immunomodulatory drugs such as lenalidomide, autologous peripheral blood stem cell transplantation or bevacizumab, an anti-VEGF monoclonal antibody. SUMMARY: Although many aspects of POEMS syndrome remain unclear, a valid biomarker of disease, VEGF, is available for diagnosis as well as a wide range of therapeutic options.