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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.
 
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

Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Dispenzieri A
Am J Hematol 2021 Jul 1;96(7):872-888. Epub 2021 May 31 doi: 10.1002/ajh.26240. PMID: 34000085
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

Etiology

Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
Dispenzieri A
Am J Hematol 2021 Jul 1;96(7):872-888. Epub 2021 May 31 doi: 10.1002/ajh.26240. PMID: 34000085
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145
Jaccard A
Hematol Oncol Clin North Am 2018 Feb;32(1):141-151. doi: 10.1016/j.hoc.2017.09.011. PMID: 29157615

Diagnosis

Masaki Y, Arita K, Sakai T, Takai K, Aoki S, Kawabata H
Ann Hematol 2022 Mar;101(3):485-490. Epub 2022 Jan 19 doi: 10.1007/s00277-022-04762-6. PMID: 35044513Free PMC Article
Dispenzieri A
Am J Hematol 2021 Jul 1;96(7):872-888. Epub 2021 May 31 doi: 10.1002/ajh.26240. PMID: 34000085
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
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
Brown R, Ginsberg L
J Neurol 2019 Jan;266(1):268-277. Epub 2018 Nov 29 doi: 10.1007/s00415-018-9110-6. PMID: 30498913Free PMC Article

Therapy

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
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
Jaccard A
Hematol Oncol Clin North Am 2018 Feb;32(1):141-151. doi: 10.1016/j.hoc.2017.09.011. PMID: 29157615
Dispenzieri A, Kourelis T, Buadi F
Hematol Oncol Clin North Am 2018 Feb;32(1):119-139. doi: 10.1016/j.hoc.2017.09.010. PMID: 29157614
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

Prognosis

Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Ali T, Qazilbash MH
Eur J Haematol 2021 Jan;106(1):14-18. Epub 2020 Oct 8 doi: 10.1111/ejh.13514. PMID: 32889731
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
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145
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

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
Sugiyama A, Yokota H, Misawa S, Mukai H, Sekiguchi Y, Koide K, Suichi T, Matsushima J, Kishimoto T, Tanei ZI, Saito Y, Ito S, Kuwabara S
BMC Neurol 2021 Jun 24;21(1):239. doi: 10.1186/s12883-021-02260-2. PMID: 34167480Free PMC Article
Khouri J, Nakashima M, Wong S
JAMA Oncol 2021 Sep 1;7(9):1383-1391. doi: 10.1001/jamaoncol.2021.0586. PMID: 34081097
Keddie S, Foldes D, Caimari F, Baldeweg SE, Bomsztyk J, Ziff OJ, Fehmi J, Cerner A, Jaunmuktane Z, Brandner S, Yong K, Manji H, Carr A, Rinaldi S, Reilly MM, D'Sa S, Lunn MP
Neurology 2020 Jul 21;95(3):e268-e279. Epub 2020 Jun 30 doi: 10.1212/WNL.0000000000009940. PMID: 32606227
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145

Recent systematic reviews

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
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
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
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
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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