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Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome

Bone Marrow Transplantation volume 43, pages 739–740 (2009)Cite this article

POEMS syndrome is a rare plasma cell proliferative disorder characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. The pathogenesis of POEMS syndrome is still not well understood, but the overproduction of vascular endothelial growth factor (VEGF) probably secreted by plasma cells is thought to be responsible.1 High-dose melphalan with Auto-SCT has been considered to be an effective strategy, but its treatment-related morbidity should be considered carefully,2 especially with its poor performance status (PS) owing to polyneuropathy and multiorgan involvement, such as cardiac, respiratory and renal failure. Novel antimyeloma agents have also been recently tried with mixed results. This report presents a severe case of POEMS syndrome, which was successfully treated by combination therapy with bevacizumab (anti-VEGF MoAb) and thalidomide, followed by Auto-SCT.

A 49-year-old male patient had developed subacute progressive sensory-motor polyneuropathy, peripheral edema, gynecomastia, skin hemangioma and skin pigmentation. CT scan revealed hepatosplenomegaly with mild pleural effusion and ascites. A small amount of monoclonal IgA-λ protein was detected, and a BM examination revealed 1.6% of plasma cells. An endocrinological examination revealed hypothyroidism and hyperprolactinemia. The patient was diagnosed to have POEMS syndrome in August 2006. He initially received VCR, doxorubicin and dexamethasone and then high-dose CY plus dexamethasone without an objective clinical benefit. He developed severe tetraplegia because of peripheral neuropathy and thereafter became totally bedridden within 4 months from the diagnosis. In December 2006, he transferred to our hospital for Auto-SCT.

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Authors and Affiliations

  1. Division of Hematology, Department of Clinical Cell Biology, Chiba University Graduate School of Medicine, Chiba, Japan

    C Ohwada, C Nakaseko, S Sakai, Y Takeda, D Abe, M Takeuchi, E Sakaida, S Masuda, N Shimizu, R Cho & M Nishimura

  2. Department of Neurology, Chiba University Graduate School of Medicine, Chiba, Japan. E-mail: vzy03227@nifty.com,

    K Kanai, S Misawa & S Kuwabara

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  1. C Ohwada
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  2. C Nakaseko
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  3. S Sakai
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  4. Y Takeda
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  11. M Nishimura
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  13. S Misawa
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Ohwada, C., Nakaseko, C., Sakai, S. et al. Successful combination treatment with bevacizumab, thalidomide and autologous PBSC for severe POEMS syndrome. Bone Marrow Transplant 43, 739–740 (2009). https://doi.org/10.1038/bmt.2008.381

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