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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: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
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: 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

POEMS syndrome: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
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
POEMS syndrome.
Keddie S, Lunn MP
Curr Opin Neurol 2018 Oct;31(5):551-558. doi: 10.1097/WCO.0000000000000610. PMID: 30138145
Paraproteinemic neuropathies.
Raheja D, Specht C, Simmons Z
Muscle Nerve 2015 Jan;51(1):1-13. Epub 2014 Nov 22 doi: 10.1002/mus.24471. PMID: 25288371

Diagnosis

POEMS syndrome: Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2023 Dec;98(12):1934-1950. Epub 2023 Sep 21 doi: 10.1002/ajh.27081. PMID: 37732822
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
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
POEMS syndrome: clinical update.
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

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: Diagnosis and Investigative Work-up.
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
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

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
Cerebral large artery stenosis and occlusion in POEMS syndrome.
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
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.
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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