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

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

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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kjh-43-145f1.tif
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 1st chemotherapy, B2 after 2nd chemotherapy and B3 after 3rd 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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