POEMS syndrome epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]

Overview

The precise incidence of POEMS syndrome is difficult to determine and we have no well established data to quote. The median age at presentation has been 51 years with the majority of the patients comprising of males. A median survival of 165 months has also been recorded. The precise incidence of POEMS syndrome is difficult to determine due to the complexity of the syndrome presentation and we have no well established data regarding the incidence. Prevalence of POEMS syndrome in Japan is reported to be about 0.3 per 100,000 individuals. There is no racial predilection to POEMS syndrome. There is no well established data regarding the gender predilection of POEMS syndrome. Data from Japan shows a male predilection of 2.5:1. The majority of POEMS syndrome cases have been reported in Japan and China.

Epidemiology and Demographics

Incidence

The precise incidence of POEMS syndrome is difficult to determine due to the complexity of the syndrome presentation and we have no well established data regarding the incidence.

Prevalence

Prevalence of POEMS syndrome in Japan is reported to be about 0.3 per 100,000 individuals.[1]

Age

The median age at presentation is 51 years.[2]

Race

There is no racial predilection to POEMS syndrome.[3][4][5]

Gender

  • There is no well established data regarding the gender predilection of POEMS syndrome.
  • Data from Japan shows a male predilection of 2.5:1.[6]

Region

The majority of POEMS syndrome cases have been reported in Japan and China.[3][4][5]

References

  1. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=2905
  2. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, Greipp PR, Witzig TE, Basu R, Suarez GA, Fonseca R, Lust JA, Gertz MA (April 2003). "POEMS syndrome: definitions and long-term outcome". Blood. 101 (7): 2496–506. doi:10.1182/blood-2002-07-2299. PMID 12456500.
  3. 3.0 3.1 Yuan M, Chen W, Zhou H, Xiao Z, Wang W, Wang W, Yin X, Xu L (2016). "Kennedy Disease Misdiagnosed as Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome: A Case Report". Med Princ Pract. 25 (3): 286–9. doi:10.1159/000442822. PMC 5588369. PMID 26618536.
  4. 4.0 4.1 Tang LM, Chen ST, Cheng SY, Lyu RK (1995). "POEMS syndrome in Chinese". Eur. Neurol. 35 (6): 349–53. doi:10.1159/000117159. PMID 8591803.
  5. 5.0 5.1 Romas E, Storey E, Ayers M, Byrne E (July 1992). "Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia". Pathology. 24 (3): 217–20. PMID 1437299.
  6. https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=2905

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