{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T13:34:32Z","timestamp":1787664872305,"version":"build-2736575974"},"reference-count":21,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2023,11,10]],"date-time":"2023-11-10T00:00:00Z","timestamp":1699574400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Italian Ministry of Health\u2014current research IRCCS"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diseases"],"abstract":"<jats:p>POEMS syndrome\u2014characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes\u2014is an uncommon and complex paraneoplastic disorder encompassing a diverse array of symptoms. Here we report the challenging case of a 34-year-old female who sought medical attention at the emergency department due to distal lower limb weakness. She was breastfeeding her first child at that time. Her condition rapidly deteriorated, making it difficult for her to perform simple tasks independently. Initially, she struggled with activities like jumping or climbing stairs. Eventually, her ability to walk was also compromised. These symptoms underscored the swift evolution of her polyneuropathy. Nerve conduction studies and electromyography confirmed a diagnosis of mixed demyelinating and axonal polyneuropathy. Subsequent investigations, including bone marrow biopsy and immunochemistry testing, revealed a plasma cell disorder characterized by lambda monoclonal gammopathy, along with elevated levels of vascular endothelial growth factor (VEGF &gt; 8000 pg\/mL). This pivotal finding led to the diagnosis of POEMS syndrome, prompting the initiation of antineoplastic therapy (daratumumab-lenalidomide-dexamethasone) to manage this condition. An autologous cell transplantation was planned. The rarity of POEMS syndrome and its diverse clinical manifestations often lead to an incorrect or delayed diagnosis. Our case underscores the importance of considering this syndrome in patients presenting with acute or subacute polyneuropathy, even if the patients are young. In conclusion, this case elucidates the diagnostic complexities of POEMS syndrome, emphasizing the integral role of comprehensive multidisciplinary evaluations and the potential influence of increased VEGF as a diagnostic key element and possible therapeutic target.<\/jats:p>","DOI":"10.3390\/diseases11040167","type":"journal-article","created":{"date-parts":[[2023,11,10]],"date-time":"2023-11-10T01:17:51Z","timestamp":1699579071000},"page":"167","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Unraveling the Neurological Complexity of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome: A Report of a Challenging Case of a Young Woman and Cutting-Edge Advancements in the Field"],"prefix":"10.3390","volume":"11","author":[{"given":"Gioconda","family":"Furciniti","sequence":"first","affiliation":[{"name":"Department of Pathophysiology and Transplantation, \u201cDino Ferrari\u201d Center, Universit\u00e0 degli Studi di Milano, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0208-0740","authenticated-orcid":false,"given":"Giuseppe","family":"Casalino","sequence":"additional","affiliation":[{"name":"Eye Clinic, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco M.","family":"Lo Russo","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1018-5139","authenticated-orcid":false,"given":"Niccol\u00f2","family":"Bolli","sequence":"additional","affiliation":[{"name":"Hematology Division, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"},{"name":"Department of Oncology and Onco-Hematology, Universit\u00e0 degli Studi di Milano, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megi","family":"Meneri","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation, \u201cDino Ferrari\u201d Center, Universit\u00e0 degli Studi di Milano, 20122 Milano, Italy"},{"name":"Neurology Unit, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giacomo P.","family":"Comi","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation, \u201cDino Ferrari\u201d Center, Universit\u00e0 degli Studi di Milano, 20122 Milano, Italy"},{"name":"Neurology Unit, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5425-969X","authenticated-orcid":false,"given":"Stefania P.","family":"Corti","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology and Transplantation, \u201cDino Ferrari\u201d Center, Universit\u00e0 degli Studi di Milano, 20122 Milano, Italy"},{"name":"Neuromuscular and Rare Diseases Unit, Department of Neuroscience, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1837-2788","authenticated-orcid":false,"given":"Daniele","family":"Velardo","sequence":"additional","affiliation":[{"name":"Neurology Unit, Foundation IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,11,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"951","DOI":"10.1002\/ajh.24171","article-title":"POEMS Syndrome: Update on Diagnosis, Risk-stratification, and Management","volume":"90","author":"Dispenzieri","year":"2015","journal-title":"Am. J. Hematol."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Bou Zerdan, M., George, T.I., Bunting, S.T., and Chaulagain, C.P. (2022). Recent Advances in the Treatment and Supportive Care of POEMS Syndrome. JCM, 11.","DOI":"10.3390\/jcm11237011"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.blre.2007.07.004","article-title":"POEMS Syndrome","volume":"21","author":"Dispenzieri","year":"2007","journal-title":"Blood Rev."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2496","DOI":"10.1182\/blood-2002-07-2299","article-title":"POEMS Syndrome: Definitions and Long-Term Outcome","volume":"101","author":"Dispenzieri","year":"2003","journal-title":"Blood"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"101064","DOI":"10.1016\/j.ajoc.2021.101064","article-title":"Asymmetric Optic Disc Edema in a Young Patient with POEMS: A Rare Presentation of a Rare Disease","volume":"22","author":"Mahallati","year":"2021","journal-title":"Am. J. Ophthalmol. Case Rep."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1136\/jnnp.2010.205369","article-title":"POEMS Syndrome with Guillan-Barre Syndrome-like Acute Onset: A Case Report and Review of Neurological Progression in 30 Cases","volume":"82","author":"Isose","year":"2011","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_7","first-page":"379","article-title":"POEMS Syndrome in a 20-Year-Old Patient Diagnosed Following a Complaint of Reduced Visual Acuity","volume":"68","author":"Doi","year":"2014","journal-title":"Acta Medica Okayama"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1016\/S0002-9394(98)00106-8","article-title":"POEMS Syndrome: An Unusual Cause of Bilateral Optic Disk Swelling","volume":"126","author":"Wong","year":"1998","journal-title":"Am. J. Ophthalmol."},{"key":"ref_9","first-page":"531","article-title":"Bilateral papilloedema as the initial symptom of POEMS syndrome","volume":"43","year":"2006","journal-title":"Rev. Neurol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"778","DOI":"10.1016\/j.ophtha.2010.08.013","article-title":"Ocular Findings in Patients with Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome","volume":"118","author":"Kaushik","year":"2011","journal-title":"Ophthalmology"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1186\/s13023-020-01392-x","article-title":"Reliability of Optic Disc Edema Area in Estimating the Severity of Papilledema in Patients with POEMS Syndrome","volume":"15","author":"Liu","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1428","DOI":"10.3389\/fimmu.2019.01428","article-title":"Characteristics of 1946 Cases of POEMS Syndrome in Chinese Subjects: A Literature-Based Study","volume":"10","author":"Wang","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1007\/s12288-013-0280-1","article-title":"Pure Motor Axonal Neuropathy, Organomegaly, Impaired Glucose Tolerance, M Protein, Skin Changes, Multiple Plasmacytomas & Acute Interstitial Nephritis in Osteolytic Myeloma: Beyond POEMS!","volume":"30","author":"Pal","year":"2014","journal-title":"Indian J. Hematol. Blood Transfus."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.jns.2011.09.014","article-title":"POEMS Syndrome with Prominent Acute Axonal Lesions","volume":"313","author":"Mathis","year":"2012","journal-title":"J. Neurol. Sci."},{"key":"ref_15","first-page":"7467","article-title":"Multimodal Imaging and Clinical Characteristics of Bone Lesions in POEMS Syndrome","volume":"8","author":"Shi","year":"2015","journal-title":"Int. J. Clin. Exp. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e2294","DOI":"10.7717\/peerj.2294","article-title":"Bone Lesions in Chinese POEMS Syndrome Patients: Imaging Characteristics and Clinical Implications","volume":"4","author":"Wang","year":"2016","journal-title":"PeerJ"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1136\/jnnp.2007.135681","article-title":"Neuropathic Pain Correlates with Myelinated Fibre Loss and Cytokine Profile in POEMS Syndrome","volume":"79","author":"Koike","year":"2008","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/j.clml.2023.04.008","article-title":"POEMS Syndrome-Clinical Picture and Management. Current Knowledge","volume":"23","author":"Jurczyszyn","year":"2023","journal-title":"Clin. Lymphoma Myeloma Leuk."},{"key":"ref_19","first-page":"826","article-title":"Hematologic Disorders and the Nervous System","volume":"29","author":"Mauermann","year":"2023","journal-title":"Continuum"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e7004","DOI":"10.1002\/ccr3.7004","article-title":"Successful Treatment with Bortezomib for POEMS Syndrome, Overcoming Complicated Severe Heart Block","volume":"11","author":"Nakamura","year":"2023","journal-title":"Clin. Case Rep."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1186\/s13045-018-0672-7","article-title":"Anti-BCMA CAR-T Cells for Treatment of Plasma Cell Dyscrasia: Case Report on POEMS Syndrome and Multiple Myeloma","volume":"11","author":"Xu","year":"2018","journal-title":"J. Hematol. Oncol."}],"container-title":["Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2079-9721\/11\/4\/167\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:20:49Z","timestamp":1760131249000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2079-9721\/11\/4\/167"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,10]]},"references-count":21,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2023,12]]}},"alternative-id":["diseases11040167"],"URL":"https:\/\/doi.org\/10.3390\/diseases11040167","relation":{},"ISSN":["2079-9721"],"issn-type":[{"value":"2079-9721","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,10]]}}}