Abstract | DISEASE OVERVIEW:
POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. DIAGNOSIS: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. RISK STRATIFICATION: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. RISK-ADAPTED THERAPY:
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Authors | Angela Dispenzieri |
Journal | American journal of hematology
(Am J Hematol)
Vol. 90
Issue 10
Pg. 951-62
(Oct 2015)
ISSN: 1096-8652 [Electronic] United States |
PMID | 26331353
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Copyright | © 2015 Wiley Periodicals, Inc. |
Topics |
- Humans
- Neoplasms, Plasma Cell
(diagnosis, metabolism, pathology, therapy)
- POEMS Syndrome
(diagnosis, metabolism, pathology, therapy)
- Risk Assessment
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