A review of POEMS syndrome.

POEMS syndrome is a rare paraneoplastic syndrome that is caused by an underlying plasma cell disorder. Its main features include polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Other important characteristics of POEMS include papilledema, extravascular volume overload, sclerotic bone lesions, and thrombocytosis/erythrocytosis (PEST). Vascular endothelial growth factor (VEGF) appears to play an important role in the disease and is especially useful for monitoring therapy, but it is not likely the sole factor driving the disease. The most commonly used therapies for POEMS include alkylators and steroids, high-dose chemotherapy with peripheral blood stem cell transplantation, lenalidomide, and bortezomib. The role of anti-VEGF antibodies is uncertain. In general, patients have an excellent prognosis if the diagnosis is made early and appropriate therapy is applied.