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KOS 2007: What’s New About the POEMS Syndrome?
By Angela Dispenzieri, MD
Angela Dispenzieri, MD
Mayo Clinic Cancer Center
Rochester, MN, USA

Mayo Clinic Cancer Center
Rochester, MN, USA


POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S), papilledema, edema, effusions, ascites, and thrombocytosis.(1,2) Virtually all patients will have either at least one sclerotic bone lesion, an elevation in plasma levels of vascular endothelial growth factor (VEGF), or co-existent Castleman’s disease. Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity. Other names for the syndrome include osteosclerotic myeloma, Crow-Fukase Syndrome, or Takatsuki syndrome. Though the pathophysiologic mechanism is not well understood, there is a correlation between treating the underlying clonal plasmaproliferative disorder and clinical improvement. This observation clearly links the plasma cell clone to the peripheral neuropathy and other clinical features, though the mechanism is not yet fully elucidated. Pro-angiogenic and pro-inflammatory cytokines have been shown to track with disease course, and VEGF is considered to be the best putative candidate for underlying pathogenesis.(3,4) Emerging cytokine and molecular data, including cytogenetic findings will be reviewed.(5)

Because a progressive neuropathy is often the dominant feature of the disease, patients are often labeled as having chronic inflammatory demyelinating polyneuropathy (CIDP). Clues that differentiate CIDP from POEMS syndrome are the presence of other paraneoplastic features of the acronym/syndrome and a lack of response to standard CIDP therapies, i.e. intravenous gammaglobulin and plasmapheresis. Instead, the mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high dose chemotherapy with peripheral blood stem cell transplant.(6) Data on novel therapies like bevacizumab and immunomodulatory therapies will be discussed.(7)

1. Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine. 1980;59:311-322.
2. Takatsuki K, Sanada I. Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol. 1983;13:543-555.
3. Gherardi RK, Belec L, Soubrier M, et al. Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. Blood. 1996;87:1458-1465.
4. Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle & Nerve. 1998;21:1390-1397.
5. Bryce AH, Ketterling RP, Gertz MA, et al. Cytogenetic analysis using multiple myeloma targets in POEMS syndrome. Proceedings of American Society of Oncology Meeting. Chicago, IL; 2007.
6. Dispenzieri A. POEMS Syndrome. Hematology (Am Soc Hematol Educ Program). 2005:360-367.
7. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood. 2005;106:1135.

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