Advanced POEMS syndrome treated with high-dose melphalan followed by autologous blood stem cell transplantation: a single-center experience

Il-Young Jang; Dok Hyun Yoon; Shin Kim; Kyoungmin Lee; Kwang-Kuk Kim; Young-Min Lim; Won-Ki Min; Cheolwon Suh

doi:10.5045/br.2014.49.1.42

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Original Article

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Blood Res 2014; 49(1):

Published online March 31, 2014

https://doi.org/10.5045/br.2014.49.1.42

Advanced POEMS syndrome treated with high-dose melphalan followed by autologous blood stem cell transplantation: a single-center experience

Il-Young Jang^1,#, Dok Hyun Yoon^1,2,#, Shin Kim², Kyoungmin Lee², Kwang-Kuk Kim³, Young-Min Lim³, Won-Ki Min⁴, and Cheolwon Suh^1,2*

Author Info. +

¹Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

²Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

³Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

⁴Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to : Correspondence to Cheolwon Suh, M.D., Ph.D. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3209, Fax: +82-2-3010-6961, csuh@amc.seoul.kr

Received: November 19, 2013; Revised: January 22, 2014; Accepted: February 19, 2014

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

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Abstract

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 transplantation 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 hematologic 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.

Keywords POEMS syndrome, Stem cell transplantation, Chemotherapy, Severity, Illness index

Article

Original Article

Blood Res 2014; 49(1): 42-48

Published online March 31, 2014 https://doi.org/10.5045/br.2014.49.1.42

Advanced POEMS syndrome treated with high-dose melphalan followed by autologous blood stem cell transplantation: a single-center experience

Il-Young Jang^1,#, Dok Hyun Yoon^1,2,#, Shin Kim², Kyoungmin Lee², Kwang-Kuk Kim³, Young-Min Lim³, Won-Ki Min⁴, and Cheolwon Suh^1,2*

¹Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

²Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

³Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

⁴Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to:Correspondence to Cheolwon Suh, M.D., Ph.D. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3209, Fax: +82-2-3010-6961, csuh@amc.seoul.kr

Received: November 19, 2013; Revised: January 22, 2014; Accepted: February 19, 2014

Abstract

Background

Methods

Results

Conclusion

High-dose melphalan followed by autologous stem cell transplantation can be considered a valid treatment option even for patients with advanced POEMS syndrome.

Keywords: POEMS syndrome, Stem cell transplantation, Chemotherapy, Severity, Illness index

Abstract

Fig 1.

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Figure 1.

Clinical features of the 9 patients with POEMS syndrome at diagnosis.

Blood Research 2014; 49: 42-48https://doi.org/10.5045/br.2014.49.1.42

Fig 2.

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Figure 2.

Kaplan-Meier curve for overall survival.

Blood Research 2014; 49: 42-48https://doi.org/10.5045/br.2014.49.1.42

Fig 3.

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Figure 3.

ECOG performance status at baseline and after ASCT (at last follow-up).

Blood Research 2014; 49: 42-48https://doi.org/10.5045/br.2014.49.1.42

Table 1 . Patient characteristics of 9 POEMS patients..

Table 2 . Medical history of each patient before high-dose melphalan followed by ASCT..

^a)Delayed for the patient's financial reasons. ^b)These patients stopped chemotherapy early due to vincristine-related neuropathy..

Abbreviations: ASCT, Autologous stem cell transplantation; C, cycle; MP, melphalan and prednisolone; SD, stable disease; Thal/Dex, thalidomide and dexamethasone; VAD, vincristine, adriamycin and dexamethasone; VGPR, very good partial response..

Table 3 . Hematologic responses, ECOG performance score, and clinical responses as changes in daily life activities..

Abbreviations: ASCT, Autologous stem cell transplantation; CR, complete response; NA, not available; NCV, nerve conduction velocity test; PD, progressive disease; PNP, peripheral neuropathy; PR, partial response; PS, performance status; sCR, stringent complete response; VGPR, very good partial response..