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Durable responses with autologous hematopoietic SCT in patients with POEMS syndrome

Bone Marrow Transplantation volume 49, pages 465–466 (2014)Cite this article

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POEMS syndrome, also known as osteosclerotic myeloma, is a constellation of polyneuropathy, monoclonal plasma cell proliferative disorder and several other clinical features.1, 2 The three major diagnostic criteria include Castleman disease, sclerotic bone lesions and elevated serum levels of vascular endothelial growth factor. The rest of the acronym, that is, organomegaly, endocrinopathy and skin changes, form the minor criteria along with extravascular volume overload, papilledema and thrombocytosis/polycythemia. Diagnosis of POEMS syndrome depends on the presence of polyneuropathy, monoclonal gammopathy and one other major and one minor criterion.3

Treatment is aimed at eradicating the underlying plasma cell clone and the control of major symptoms. In cases of 1–3 plasmacytomas without BM infiltration, localized radiation therapy may be sufficient.4 Conversely, widespread disease requires systemic therapy, which is adapted largely from therapy for multiple myeloma.4 Owing to its rarity, current literature for POEMS syndrome comprises case reports and series, and no randomized trials have been conducted.5 In 2001, the first report of a successful autologous hematopoietic SCT (auto-HCT) in a patient with POEMS syndrome was published,6 followed by similar case series from different centers.7, 8, 9 Results suggest that high-dose chemotherapy and auto-HCT are associated with durable clinical response, but with significant post-transplant morbidity.7, 8, 9 Here we present our experience in seven patients with POEMS syndrome who underwent auto-HCT at the MD Anderson Cancer Center.

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Authors and Affiliations

  1. Hematology Oncology Fellowship, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    K Patel

  2. The University of Texas UT Health Internal Medicine Residency Program, Houston, TX, USA

    M Nusrat

  3. Stem Cell Transplantation and Cellular Therapy, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    N Shah, Q Bashir, S Parmar, R Champlin & M H Qazilbash

  4. Department of Lymphoma/Myeloma, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    J Shah, S Thomas, D Weber & R Z Orlowski

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  1. K Patel
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  10. R Champlin
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  11. M H Qazilbash
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Correspondence to K Patel.

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Patel, K., Nusrat, M., Shah, N. et al. Durable responses with autologous hematopoietic SCT in patients with POEMS syndrome. Bone Marrow Transplant 49, 465–466 (2014). https://doi.org/10.1038/bmt.2013.209

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