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KMID : 0366220140490010042
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Korean Journal of Hematology
2014 Volume.49 No. 1 p.42 ~ p.48
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Advanced POEMS syndrome treated with high-dose melphalan followed by autologous blood stem cell transplantation: a single-center experience
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Jang Il-Young
Yoon Dok-Hyun
Kim Shin
Lee Kyoung-Min
Kim Kwang-Kuk
Lim Young-Min
Min Won-Ki
Suh Cheol-Won
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Abstract
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Background: POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. However, there are minimal reports on clinical outcomes after autologous stem cell trans-plantation for patients with advanced disease and very poor performance status.
Methods: We retrospectively evaluated 9 advanced POEMS syndrome patients, who had an Eastern Cooperative Oncology Group performance status score of 3 or 4, and were treated with high-dose melphalan therapy followed by autologous stem cell transplantation from 2004 to 2011.
Results: Eight patients achieved initial hematologic response, 4 of whom had complete responses. At a median follow-up of 44 months (range, 8?94 months), 7 patients were alive, with 3-year overall survival rate of 77.8%. There were no hematologic relapses in the survivors. One patient died of disease progression; the other died of pneumonia despite a hemato-logic response 3 months after autologous stem cell transplantation. All survivors achieved improvement in general performance status and in clinical response.
Conclusion: High-dose melphalan followed by autologous stem cell transplantation can be considered a valid treatment option even for patients with advanced POEMS syndrome.
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KEYWORD
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POEMS syndrome, Stem cell transplantation, Chemotherapy, Severity, Illness index
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