Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome.
10.5045/kjh.2008.43.3.145
- Author:
Seok Hui KANG
1
;
Jong Yul LEE
;
Sang Hoon CHUN
;
Hyung Wook PARK
;
Chang Ki MIN
;
Chun Choo KIM
;
Myungshin KIM
;
Yonggoo KIM
Author Information
1. Department of Internal Medicine, St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea. ckmin@catholic.ac.kr
- Publication Type:Original Article
- Keywords:
POEMS syndrome;
Bortezomib;
Vascular endothelial growth factor;
Autologous stem cell transplantation;
Peripheral neuropathy
- MeSH:
Boronic Acids;
Cytokines;
Follow-Up Studies;
Humans;
Melphalan;
Peripheral Nervous System Diseases;
POEMS Syndrome;
Polyneuropathies;
Pyrazines;
Skin;
Skin Diseases;
Stem Cell Transplantation;
Stem Cells;
Vascular Endothelial Growth Factor A;
Bortezomib
- From:Korean Journal of Hematology
2008;43(3):145-149
- CountryRepublic of Korea
- Language:English
-
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.
