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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

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
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

Etiology

Evidence-based medical treatment of POEMS syndrome.
Khwaja J, D'Sa S, Lunn MP, Sive J
Br J Haematol 2023 Jan;200(2):128-136. Epub 2022 Aug 7 doi: 10.1111/bjh.18400. PMID: 35934319
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
CIDP mimics: a case series.
Moshe-Lilie O, Ensrud E, Ragole T, Nizar C, Dimitrova D, Karam C
BMC Neurol 2021 Feb 28;21(1):94. doi: 10.1186/s12883-021-02118-7. PMID: 33639867Free PMC Article
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.
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145

Diagnosis

Castleman disease and TAFRO syndrome.
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
Paraproteinemia and neuropathy.
Koike H, Katsuno M
Neurol Sci 2021 Nov;42(11):4489-4501. Epub 2021 Sep 16 doi: 10.1007/s10072-021-05583-7. PMID: 34529193
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
Paraneoplastic neuropathies.
Antoine JC, Camdessanché JP
Curr Opin Neurol 2017 Oct;30(5):513-520. doi: 10.1097/WCO.0000000000000475. PMID: 28682959

Therapy

Evidence-based medical treatment of POEMS syndrome.
Khwaja J, D'Sa S, Lunn MP, Sive J
Br J Haematol 2023 Jan;200(2):128-136. Epub 2022 Aug 7 doi: 10.1111/bjh.18400. PMID: 35934319
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.
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

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
POEMS syndrome: A multisystem clonal disorder.
Ali T, Qazilbash MH
Eur J Haematol 2021 Jan;106(1):14-18. Epub 2020 Oct 8 doi: 10.1111/ejh.13514. PMID: 32889731
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
European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias.
Gavriatopoulou M, Musto P, Caers J, Merlini G, Kastritis E, van de Donk N, Gay F, Hegenbart U, Hajek R, Zweegman S, Bruno B, Straka C, Dimopoulos MA, Einsele H, Boccadoro M, Sonneveld P, Engelhardt M, Terpos E
Leukemia 2018 Sep;32(9):1883-1898. Epub 2018 Jul 23 doi: 10.1038/s41375-018-0209-7. PMID: 30038381

Clinical prediction guides

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
Clinical characteristics, risk factors, and outcomes of POEMS syndrome: A longitudinal cohort study.
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
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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