title: [New treatment strategy for Crow-Fukase (POEMS) syndrome].
abstract: Crow-Fukase syndrome, also called POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome, is a rare cause of demyelinating and axonal mixed neuropathy with multiorgan involvement. The pathogenesis of Crow-Fukase syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably mediated by monoclonal proliferation of plasma cells, is likely to be responsible for most of the characteristic symptoms. There is no established treatment regimen. In appropriate candidates, high-dose chemotherapies with autologous peripheral blood stem cell transplantation is highly recommended, because this treatment could result in obvious improvement in neuropathy as well as other symptoms, with a significant decrease in serum VEGF levels. Indication of this treatment has not yet been established, and long-term prognosis is unclear at present. Thalidomide should be considered for patients who are not indicated for transplantation therapy. Treatments that should be considered as future therapy include lenalidomide, bortezomib, and anti-VEGF monoclonal antibody (bevacizumab).
authors.affiliation: Department of Neurology, Graduate School of Medicine, Chiba University.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kuwabara
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000068258:Bevacizumab; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D003131:Combined Modality Therapy; D003711:Demyelinating Diseases / Q000209:etiology; D006801:Humans; D000077269:Lenalidomide; D008558:Melphalan / Q000008:administration & dosage*; D016878:POEMS Syndrome / Q000209:etiology / Q000378:metabolism / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D011719:Pyrazines / Q000627:therapeutic use; D013792:Thalidomide / Q000031:analogs & derivatives / Q000627:therapeutic use; D014182:Transplantation, Autologous; D042461:Vascular Endothelial Growth Factor A / Q000276:immunology / Q000378:metabolism
chemical_list: D020533:Angiogenesis Inhibitors; D061067:Antibodies, Monoclonal, Humanized; D001897:Boronic Acids; D011719:Pyrazines; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab; D013792:Thalidomide; D000069286:Bortezomib; D000077269:Lenalidomide; D008558:Melphalan