{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T07:09:28Z","timestamp":1791011368078,"version":"4.1.0"},"reference-count":173,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2012,7,17]],"date-time":"2012-07-17T00:00:00Z","timestamp":1342483200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Disease overview:<\/jats:italic>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.<\/jats:p><jats:p><jats:italic>Diagnosis:<\/jats:italic>The diagnosis of POEMS syndrome is made with 3 of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.<\/jats:p><jats:p><jats:italic>Risk stratification:<\/jats:italic>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.<\/jats:p><jats:p><jats:italic>Risk\u2010adapted therapy:<\/jats:italic>For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. The benefit of anti\u2010VEGF antibodies is conflicting. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 2012. \u00a9 2012 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.23288","type":"journal-article","created":{"date-parts":[[2012,7,19]],"date-time":"2012-07-19T03:14:06Z","timestamp":1342667646000},"page":"804-814","source":"Crossref","is-referenced-by-count":61,"title":["POEMS syndrome: Update on diagnosis, risk\u2010stratification, and management"],"prefix":"10.1002","volume":"87","author":[{"given":"Angela","family":"Dispenzieri*","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,7,17]]},"reference":[{"key":"e_1_2_11_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198007000-00006"},{"key":"e_1_2_11_3_2","first-page":"543","article-title":"Plasma cell dyscrasia with polyneuropathy and endocrine disorder: Clinical and laboratory features of 109 reported cases","volume":"13","author":"Takatsuki K","year":"1983","journal-title":"Jpn J Clin Oncol"},{"key":"e_1_2_11_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.34.6.712"},{"key":"e_1_2_11_5_2","doi-asserted-by":"publisher","DOI":"10.1080\/1042819031000111044"},{"key":"e_1_2_11_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(94)90350-6"},{"key":"e_1_2_11_7_2","first-page":"229","article-title":"The clinical study of POEMS syndrome in China","volume":"31","author":"Zhang B","year":"2010","journal-title":"Neuro Endocrinol Lett"},{"key":"e_1_2_11_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-010-1149-0"},{"key":"e_1_2_11_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2010.07.124"},{"key":"e_1_2_11_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-301706"},{"key":"e_1_2_11_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2007.07.004"},{"key":"e_1_2_11_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)91261-1"},{"key":"e_1_2_11_13_2","first-page":"813","article-title":"Arterial obliteration in POEMS syndrome: Possible role of vascular endothelial growth factor","volume":"25","author":"Soubrier M","year":"1998","journal-title":"J Rheumatol"},{"key":"e_1_2_11_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199811)21:11<1390::AID-MUS5>3.0.CO;2-4"},{"key":"e_1_2_11_15_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01208.x"},{"key":"e_1_2_11_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0272-6386(99)70386-0"},{"key":"e_1_2_11_17_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.39.1101"},{"key":"e_1_2_11_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh519"},{"key":"e_1_2_11_19_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000188757.38495.23"},{"key":"e_1_2_11_20_2","doi-asserted-by":"publisher","DOI":"10.1620\/tjem.210.269"},{"key":"e_1_2_11_21_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-06-362392"},{"key":"e_1_2_11_22_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.46.6246"},{"key":"e_1_2_11_23_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-12-5045"},{"key":"e_1_2_11_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/annonc\/mdm602"},{"key":"e_1_2_11_25_2","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-03-0910"},{"key":"e_1_2_11_26_2","doi-asserted-by":"publisher","DOI":"10.1038\/bmt.2008.381"},{"key":"e_1_2_11_27_2","article-title":"Bevacizumab therapy for POEMS syndrome","volume":"107","author":"Badros A","year":"2006","journal-title":"Blood"},{"key":"e_1_2_11_28_2","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.11315"},{"key":"e_1_2_11_29_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780400430"},{"key":"e_1_2_11_30_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.41.1196"},{"key":"e_1_2_11_31_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.56.6.818"},{"key":"e_1_2_11_32_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0953-6205(01)00199-6"},{"key":"e_1_2_11_33_2","first-page":"ECR02","article-title":"Restricted use of Vlambda genes in POEMS syndrome","volume":"89","author":"Soubrier M","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_11_34_2","first-page":"2483","article-title":"Restricted oligo\u2010clonal usage of monoclonal immunoglobulin {lambda} light chain germline in POEMS syndrome","volume":"110","author":"Nakaseko C","year":"2007","journal-title":"ASH Annual Meeting Abstracts"},{"key":"e_1_2_11_35_2","first-page":"2744","article-title":"Immunoglobulin variable light chain restriction, cytokine expression and plasma cell\u2010stromal cell interactions in POEMS syndrome patients","volume":"112","author":"Aravamudan B","year":"2008","journal-title":"ASH Annual Meeting Abstracts"},{"key":"e_1_2_11_36_2","doi-asserted-by":"crossref","unstructured":"BryceAH KetterlingRP GertzMA et al. 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