Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome / 대한혈액학회지

ABSTRACT

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.