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POEMS Syndrome: an Enigma

  • Multiple Myeloma (P Kapoor, Section Editor)
  • Published:
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Abstract

POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman’s disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Treatment is based on extent of the disease. Radiotherapy is used for localized disease. Systemic therapy is required for disseminated disease, with bone marrow involvement by clonal plasma cells, or in patients who progress shortly after radiation. Upfront autologous stem cell transplantation is the treatment of choice for transplant-eligible patients. Outcomes are typically superior to that of standard myeloma. Herein, using a case vignette, we outline the latest evidence regarding the prognostication and management of POEMS syndrome, with a focus on its relapsing-remitting course.

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Authors and Affiliations

  1. Division of Hematology, Medicine and Oncology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Rahma Warsame & Prashant Kapoor

  2. Department of Hematology & Stem Cell Transplantation, Army Hospital (Research & Referral), Delhi, 110010, India

    Uday Yanamandra

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  1. Rahma Warsame
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  2. Uday Yanamandra
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Correspondence to Prashant Kapoor.

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Rahma Warsame and Uday Yanamandra each declare no potential conflicts of interest. Prashant Kapoor reports grants from Amgen, Takeda, and Celgene. Dr. Kapoor is a section editor for Current Hematologic Malignancy Reports.

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This article is part of the Topical Collection on Multiple Myeloma

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Warsame, R., Yanamandra, U. & Kapoor, P. POEMS Syndrome: an Enigma. Curr Hematol Malig Rep 12, 85–95 (2017). https://doi.org/10.1007/s11899-017-0367-0

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