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POEMS syndrome

MedGen UID:
39276
•Concept ID:
C0085404
•
Disease or Syndrome
Synonyms: Crow-Fukase syndrome; Polyneuropathy organomegaly; Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes syndrome
SNOMED CT: Polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes (79268002); POEMS - Polyneuropathy organomegaly endocrinopathy monoclonal and skin changes (79268002); POEMS syndrome (79268002); Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (79268002); Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome (79268002)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0017364
Orphanet: ORPHA2905

Definition

POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPOEMS syndrome

Professional guidelines

PubMed

POEMS Syndrome-Clinical Picture and Management. Current Knowledge.
Jurczyszyn A, Olszewska-Szopa M, Vesole D
Clin Lymphoma Myeloma Leuk 2023 Aug;23(8):575-582. Epub 2023 Apr 26 doi: 10.1016/j.clml.2023.04.008. PMID: 37210272
POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2021 Jul 1;96(7):872-888. Epub 2021 May 31 doi: 10.1002/ajh.26240. PMID: 34000085
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139

Recent clinical studies

Therapy

Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach.
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
POEMS Syndrome: Therapeutic Options.
Jaccard A
Hematol Oncol Clin North Am 2018 Feb;32(1):141-151. doi: 10.1016/j.hoc.2017.09.011. PMID: 29157615
POEMS syndrome.
Nyunt WWT, Remli R, Abdul Muttlib FA, Leong CF, Masir N, Tumian NR, Abdul Wahid SFS
Malays J Pathol 2017 Dec;39(3):297-303. PMID: 29279594
POEMS syndrome.
Dispenzieri A
Blood Rev 2007 Nov;21(6):285-99. Epub 2007 Sep 11 doi: 10.1016/j.blre.2007.07.004. PMID: 17850941

Prognosis

POEMS Syndrome-Clinical Picture and Management. Current Knowledge.
Jurczyszyn A, Olszewska-Szopa M, Vesole D
Clin Lymphoma Myeloma Leuk 2023 Aug;23(8):575-582. Epub 2023 Apr 26 doi: 10.1016/j.clml.2023.04.008. PMID: 37210272
Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Castleman Disease.
Wang W, Medeiros LJ
Surg Pathol Clin 2019 Sep;12(3):849-863. Epub 2019 May 23 doi: 10.1016/j.path.2019.03.003. PMID: 31352991
POEMS syndrome.
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145
POEMS Syndrome: an Enigma.
Warsame R, Yanamandra U, Kapoor P
Curr Hematol Malig Rep 2017 Apr;12(2):85-95. doi: 10.1007/s11899-017-0367-0. PMID: 28299525

Clinical prediction guides

Gammopathic dermopathy: characterization of cutaneous MGUS.
Gordon ER, Chen C, Trager MH, Adeuyan O, Lapolla BA, Fahmy LM, Schreidah CM, Wetter DA, Geskin LJ
Leuk Lymphoma 2024 Jul;65(7):989-996. Epub 2024 Apr 6 doi: 10.1080/10428194.2024.2336046. PMID: 38581379
Clinical and neurophysiological description of patients with POEMS syndrome.
Lee-Chen L, Williams-de-Roux R, Chiquete E, Aceves-Buendía JJ, Ruiz-Ruiz E, Portillo-Valle J, Bliskunova T, Rodríguez-Perea E, Toapanta-Yanchapaxi L, García-Ramos G, Cantú-Brito C, Estañol B
Gac Med Mex 2021;157(5):466-472. doi: 10.24875/GMM.M21000600. PMID: 35104271
Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review.
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Prevalence and Course of Endocrinopathy in POEMS Syndrome.
Caimari F, Keddie S, Lunn MP, D'Sa S, Baldeweg SE
J Clin Endocrinol Metab 2019 Jun 1;104(6):2140-2146. doi: 10.1210/jc.2018-01516. PMID: 30239770
POEMS syndrome.
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145

Recent systematic reviews

Efficacy and Safety of Regimens Used for the Treatment of POEMS Syndrome- A Systematic Review.
Faizan U, Sana MK, Farooqi MS, Hashmi H
Clin Lymphoma Myeloma Leuk 2022 Jan;22(1):e26-e33. Epub 2021 Aug 2 doi: 10.1016/j.clml.2021.07.033. PMID: 34507924
Neuropsychiatric manifestations in inflammatory neuropathies: A systematic review.
Rajabally YA, Seri S, Cavanna AE
Muscle Nerve 2016 Jun;54(1):1-8. Epub 2016 May 10 doi: 10.1002/mus.25112. PMID: 26999767Free PMC Article
Lenalidomide in patients with POEMS syndrome: a systematic review and pooled analysis.
Zagouri F, Kastritis E, Gavriatopoulou M, Sergentanis TN, Psaltopoulou T, Terpos E, Dimopoulos MA
Leuk Lymphoma 2014 Sep;55(9):2018-23. Epub 2014 Feb 17 doi: 10.3109/10428194.2013.869329. PMID: 24295131
Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome.
Kuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C
Cochrane Database Syst Rev 2012 Jun 13;2012(6):CD006828. doi: 10.1002/14651858.CD006828.pub3. PMID: 22696361Free PMC Article
Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome.
Kuwabara S, Dispenzieri A, Arimura K, Misawa S
Cochrane Database Syst Rev 2008 Oct 8;(4):CD006828. doi: 10.1002/14651858.CD006828.pub2. PMID: 18843731

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