Crow-Fukase (POEMS) syndrome: pathophysiology and treatments

POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare multiorgan disorder associated with plasma cell dyscrasia and overproduction of vascular endothelial growth factor (VEGF). VEGF presumably plays an important role in the pathogenesis of the syndrome by its strong action on neo-vasculization and increased vascular permeability. POEMS syndrome is potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy and/or massive peripheral edema or pleural effusion/ascites. There is no established treatment regimen. In appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation is recommended, because this treatment could result in obvious improvement in neuropathy as well as other symptoms, with a significant decrease in serum VEGF levels. However, from pooled data, the transplant-related mortality is reported to be 5%, and there is a risk of relapse several years later. Treatments that should be considered as future therapy include thalidomide or lenalidomide, and anti-VEGF monoclonal antibody (bevacizumab).