title: Successful treatment of POEMS syndrome with bortezomib and dexamethasone, combined with radiotherapy, and followed by autologous stem cell transplantation.
abstract: POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. High-dose therapy (HDT) and autologous stem cell transplantation (ASCT) are an effective therapy, but optimal treatment options are still under debate. Bortezomib is an important agent for the treatment of patients with multiple myeloma and has recently been reported as efficacious in the treatment of patients with POEMS syndrome. We present a case of POEMS syndrome in a 33-year-old woman, who was successfully treated with BorDex (bortezomib and dexamethasone) combined with radiotherapy, and followed by ASCT. She was diagnosed with POEMS syndrome with a localized plasmacytoma of bone 5 months after her initial symptoms of heart failure. Her Eastern Cooperative Oncology Group (ECOG) performance status was 4. She was first administered BorDex therapy, which was subsequently combined with radiotherapy. Her general condition including heart failure dramatically improved after four cycles of BorDex therapy and radiation, resulting in partial response. After chemoradiotherapy, HDT and ASCT were performed. After treatment, she was able to walk unassisted and her plasma endothelial growth factor (VEGF) level decreased. She did not experience neurotoxicity induced by bortezomib. Bortezomib was well tolerated and we suggest that BorDex therapy followed by HDT and ASCT may be an effective therapy for POEMS syndrome.
authors.affiliation: Department of Internal Medicine and Clinical Immunology, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa, Yokohama, 236-0004, Japan, yoshimijuly@yahoo.co.jp.
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Ishii
authors.forename: Etsuko
authors.initials: E
authors.lastname: Yamazaki
authors.forename: Yasufumi
authors.initials: Y
authors.lastname: Ishiyama
authors.forename: Eri
authors.initials: E
authors.lastname: Yamamoto
authors.forename: Yukako
authors.initials: Y
authors.lastname: Hattori
authors.forename: Maki
authors.initials: M
authors.lastname: Hagihara
authors.forename: Naoto
authors.initials: N
authors.lastname: Tomita
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Ishigatsubo
mesh_terms: D000328:Adult; D001897:Boronic Acids / Q000627:therapeutic use*; D000069286:Bortezomib; D003907:Dexamethasone / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016878:POEMS Syndrome / Q000175:diagnosis / Q000628:therapy*; D049268:Positron-Emission Tomography; D011719:Pyrazines / Q000627:therapeutic use*; D011878:Radiotherapy; D015898:Tomography Scanners, X-Ray Computed; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D003907:Dexamethasone