[POEMS Syndrome]

Abstract

POEMS syndrome is a systemic disease characterized by monoclonal plasma cell proliferation and the overproduction of the vascular endothelial growth factor. Japanese researchers have made significant contributions to the establishment of disease concepts and the development of treatments. Although many aspects of the pathophysiology remain unclear, therapies that target plasma cells have the potential to markedly improve the prognosis of affected individuals. However, the only drug currently approved for the treatment of this syndrome is thalidomide, which was authorized in 2021. Numerous challenges need to be addressed to further improve patient outcomes.