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Current Hematologic Malignancy Reports
Published in: Current Hematologic Malignancy Reports 2/2017

01-04-2017 | Multiple Myeloma (P Kapoor, Section Editor)

POEMS Syndrome: an Enigma

Authors: Rahma Warsame, Uday Yanamandra, Prashant Kapoor

Published in: Current Hematologic Malignancy Reports | Issue 2/2017

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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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Metadata
Title
POEMS Syndrome: an Enigma
Authors
Rahma Warsame
Uday Yanamandra
Prashant Kapoor
Publication date
01-04-2017
Publisher
Springer US
Published in
Current Hematologic Malignancy Reports / Issue 2/2017
Print ISSN: 1558-8211
Electronic ISSN: 1558-822X
DOI
https://doi.org/10.1007/s11899-017-0367-0

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