POEMS syndrome is a unique clinical entity defined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and other paraneoplastic features such as organomegaly, endocrinopathy, skin changes, papilledema, sclerotic bone lesions, extravascular volume overload. Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity. Other names for the syndrome include ostesclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome. The median survival of patients with POEMS syndrome is superior to those with multiple myeloma (165 and 38 months, respectively), independent of the number of presenting features, bone lesions, or plasma cells at diagnosis. There is no standard treatment for this disorder, the mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high-dose chemotherapy with peripheral blood stem cell transplantation.
POEMS syndrome; polyneuropathy; monoclonal protein; plasma cell disorder; endocrinopathy
