Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome

Seok-Hui Kang; Jong-Yul Lee; Sang-Hoon Chun; Hyung-Wook Park; Chang-Ki Min; Chun-Choo Kim; Myungshin Kim; Yonggoo Kim

doi:10.5045/kjh.2008.43.3.145

Journal List > Korean J Hematol > v.43(3) > 1032776

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Kang, Lee, Chun, Park, Min, Kim, Kim, and Kim: Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome

Original Article

Korean J Hematol 2008;43(3):145-149.

Published online: 19 September 2008

DOI: https://doi.org/10.5045/kjh.2008.43.3.145

Bortezomib Therapy Followed by Autologous Stem Cell Transplantation in POEMS Syndrome

Seok-Hui Kang, M.D.¹, Jong-Yul Lee, M.D.¹, Sang-Hoon Chun, M.D.¹, Hyung-Wook Park, M.D.¹, Chang-Ki Min, M.D.^1,, Chun-Choo Kim, M.D.¹, Myungshin Kim, M.D.², Yonggoo Kim, M.D.²

¹Department of Internal Medicine, Seoul, Korea

²Department of Hospital Pathology, St. Mary's Hospital, The Catholic University of Korea, Seoul, Korea

Correspondence to：Chang-Ki Min, M.D. Department of Internal Medicine, St. Mary's Hospital, The Catholic University of Korea 62, Yeouido-dong, Yeongdeungpo-gu, Seoul 150-713, Korea Tel: ＋82-2-3779-1124, Fax: ＋82-2-780-3132 E-mail: ckmin@catholic.ac.kr

Received 13 May 2008 Revised 25 August 2008 Accepted 29 August 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

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Keywords: POEMS syndrome, Bortezomib, Vascular endothelial growth factor, Autologous stem cell transplantation, Peripheral neuropathy

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Fig. 1

Serial changes in serum levels of M-protein (A) and vascular endothelial growth factor (B) following bortezomib treatment and autologuos stem cell transplantation (ASCT). Abbreviations: B1 after 1^st chemotherapy, B2 after 2^nd chemotherapy and B3 after 3^rd chemotherapy, prior to ASCT (preASCT), and after ASCT (postASCT).

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Table 1.

Characteristics of patients with POEMS syndrome

Patient	Age (y)	Sex	Polyneuropathy	Organomeg	aly Endocrinopathy	M component	Skin changes
1	40	M	Sensorimotor, 4 limbs	Spleen	(−)	IgG (lambda)	Hyperpigmentation
2	55	F	Sensorimotor, 4 limbs	Spleen	(−)	IgA (lambda)	(−)
3	56	F	Sensorimotor, 4 limbs	(−)	Diabetes mellitus	IgG (lambda)	Hyperpigmentation

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