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POEMS syndrome treated with melphalan high-dose therapy and autologous blood stem cell transplantation: a single-institution experience

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Abstract

The acronym POEMS syndrome stands for a rare multi-system disorder, comprised of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Here, we present a single-center report of a series of five POEMS patients treated with melphalan high-dose therapy (HDT) with subsequent autologous blood stem cell transplantation (ABSCT). After a median follow-up of 52 months from time of diagnosis (range, 15–192) and a median follow-up of 18 months after ABSCT (range, 11–120), all patients were alive. Overall, no severe transplantation-associated complications such as engraftment syndrome or peri- or post-transplant death were noted. In two cases, HDT followed by ABSCT resulted in a complete hematologic response; in the additional three cases, partial responses (PR) were achieved including one very good hematologic PR. Only one patient with initial PR developed progressive disease nearly 2.5 years after transplantation. Consequently, a second HDT with ABSCT was successfully applied resulting in clinical improvement and hematologic PR. In line with previous single-center reports, melphalan HDT followed by ABSCT proved to be a first-line treatment option with tolerable side effects in severely affected POEMS patients with progressing symptoms.

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Authors and Affiliations

  1. Department of Medicine, Hematology/Oncology, University of Muenster, Muenster, Germany

    Gabriela B. Thoennissen, Nils H. Thoennissen, Andreas Hilbig, Andrea Kerkhoff, Ruediger Liersch, Utz Krug, Steffen Koschmieder, Carsten Müller-Tidow, Rolf Mesters, Martin Kropff & Wolfgang E. Berdel

  2. Department of Medical Informatics, University of Muenster, Muenster, Germany

    Fleur Fritz

  3. Department of Medicine, Oncology/Hematology/SCT, University of Aachen, Aachen, Germany

    Steffen Koschmieder

  4. Department of Medicine A, Hematology and Oncology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149, Muenster, Germany

    Gabriela B. Thoennissen

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  1. Gabriela B. Thoennissen
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  2. Nils H. Thoennissen
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  3. Fleur Fritz
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  4. Andreas Hilbig
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Correspondence to Gabriela B. Thoennissen.

Additional information

Gabriela B. Thoennissen and Nils H. Thoennissen contributed equally and should be considered first authors.

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Thoennissen, G.B., Thoennissen, N.H., Fritz, F. et al. POEMS syndrome treated with melphalan high-dose therapy and autologous blood stem cell transplantation: a single-institution experience. Ann Hematol 91, 1419–1425 (2012). https://doi.org/10.1007/s00277-012-1473-7

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  • DOI: https://doi.org/10.1007/s00277-012-1473-7

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