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KMID : 0366220080430030145
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Korean Journal of Hematology
2008 Volume.43 No. 3 p.145 ~ p.149
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Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome
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Kang Seok-Hui
Kim Myung-Shin
Chun Sang-Hoon
Lee Jong-Yul
Kim Yong-Goo
Park Hyung-Wook
Min Chang-Ki
Kim Chun-Choo
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Abstract
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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.
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KEYWORD
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POEMS syndrome, Bortezomib, Vascular endothelial growth factor, Autologous stem cell transplantation, Peripheral neuropathy
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