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.