{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T12:29:45Z","timestamp":1789475385820,"version":"build-2803163510"},"reference-count":12,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2022,4,15]],"date-time":"2022-04-15T00:00:00Z","timestamp":1649980800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background: POEMS syndrome (POEMS) is a rare plasma cell clonal paraneoplastic syndrome consisting of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes presenting with idiopathic multicentric Castleman disease (iMCD) histology, the treatment of which has not yet been well established. iMCD is also a distinctive rare non-clonal lymphoproliferative disorder, of which dramatic response to Siltuximab, a monoclonal anti-IL-6 antibody, has been reported recently. Methods: the differential diagnosis between POEMS and iMCD can be very challenging because of the identical histology, overlapping similar symptoms such as polyneuropathy, and vital signs insidiously presented to diagnose POEMS. Results: here, we report the case of a 53-year-old man with iMCD treated for 8 years developing sequential polyneuropathy, monoclonal gammopathy, and bone lytic lesions, all of which were confirmed after his iMCD achieved complete remission resulting from siltuximab administration and finally confirmed as POEMS. Conclusions: we describe the clinical ambiguity of disease presenting that we can face in the real world between iMCD and POEMS and emphasise careful, enduring observation lasting several years.<\/jats:p>","DOI":"10.3390\/diagnostics12040998","type":"journal-article","created":{"date-parts":[[2022,4,16]],"date-time":"2022-04-16T07:42:41Z","timestamp":1650094961000},"page":"998","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["POEMS Syndrome: Presented as Idiopathic Multicentric Castleman Disease of Plasma Cell Variant for Eight Years and Dramatic Treatment with Siltuximab Followed by Autologous Peripheral Blood Stem Cell Transplantation"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4302-9263","authenticated-orcid":false,"given":"Yong-Moon","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Pathology, College of Medicine, Dankook University, Cheonan 31116, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0054-3641","authenticated-orcid":false,"given":"Yoon Seok","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Hematology-Oncology, College of Medicine, Ajou University, Suwon 16499, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin-Man","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Pathology, College of Medicine, Chungnam National University, 266 Munwha-ro, Jung-gu, Daejeon 35015, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,4,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1002\/ajh.25495","article-title":"POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management","volume":"94","author":"Dispenzieri","year":"2019","journal-title":"Am. 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Psychiatry"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1200\/JCO.1985.3.9.1202","article-title":"A systemic lymphoproliferative disorder with morphologic features of Castleman\u2019s disease: Clinical findings and clinicopathologic correlations in 15 patients","volume":"3","author":"Frizzera","year":"1985","journal-title":"J. Clin. Oncol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1093\/ajcp\/83.1.27","article-title":"Multicentric angiofollicular lymph node hyperplasia (Castleman\u2019s disease) followed by Kaposi\u2019s sarcoma in two homosexual males with the acquired immunodeficiency syndrome (AIDS)","volume":"83","author":"Lachant","year":"1985","journal-title":"Am. J. Clin. 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Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.hoc.2017.09.012","article-title":"The Peripheral Neuropathies of POEMS Syndrome and Castleman Disease","volume":"32","author":"Mauermann","year":"2018","journal-title":"Hematol. Clin. N. Am."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1097\/00000658-195803000-00018","article-title":"Localized mediastinal lymph-node hyperplasia resembling thymoma; a case report","volume":"147","author":"Inada","year":"1958","journal-title":"Ann. Surg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"215","DOI":"10.3109\/03009749409103064","article-title":"Mixed connective tissue disease associated with multicentric Castleman\u2019s disease","volume":"23","author":"Nanki","year":"1994","journal-title":"Scand. J. Rheumatol."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/4\/998\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:54:41Z","timestamp":1760136881000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/4\/998"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,15]]},"references-count":12,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["diagnostics12040998"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics12040998","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,15]]}}}