Background: The acronym POEMS refers to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Increased levels of cytokines, including vascular endothelial growth factor (VEGF), appear to play a pathogenic role. POEMS syndrome is progressive and eventually leads to death from neurological problem without therapy. Methods: We treated 3 patients affected by POEMS syndrome with front-line bortezomib treatment and the high-dose melphalan with autologous stem cell transplantation (ASCT). Results: Bortezomib reduced circulating levels of VEGF in sera. After a median follow-up of 18 months (range, 16∼20), all patients are alive with progressive improvement in neurological disease, skin changes, performance status and have no evidence of clonal plasmacytosis or organomegaly. Conclusion: ASCT following bortezomib treatment may be a potential treatment option for patients with POEMS syndrome. (Korean J Hematol 2008;43:145-149.)

Keywords: POEMS syndrome, Bortezomib, Vascular endothelial growth factor, Autologous stem cell transplantation, Peripheral neuropathy