Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey

Tomoki Suichi, Sonoko Misawa, Minako Beppu, Sho Takahashi, Yukari Sekiguchi, Kazumoto Shibuya, Hiroshi Amino, Atsuko Tsuneyama, Yo Ichi Suzuki, Keigo Nakamura, Yasunori Sato, Satoshi Kuwabara

Research output: Contribution to journalArticle

Abstract

OBJECTIVE: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. METHODS: We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. RESULTS: The estimated number of patients with POEMS was 392 (95% confidence interval [CI] 320-464), and the prevalence was 0.3 per 100,000. Detailed clinical profiles were available for 167 patients. Median age at onset was 54 years (range, 21-84 years), and the ratio of male to female was 1.5. All patients showed polyneuropathy; 89% had monoclonal plasma cell proliferation; and 84% had elevated vascular endothelial growth factor level in whom pretreatment serum or plasma was available (n = 87). Other common features were skin changes (84%), edema/effusion (81%), and organomegaly (76%). A total of 160 patients were treated with any of the following: radiation, corticosteroids, melphalan, thalidomide, lenalidomide, bortezomib, or autologous stem cell transplantation. Primary therapeutic options were thalidomide (n = 86) and autologous stem cell transplantation (n = 71). Thirty-nine patients (24%) were initially treated with corticosteroid alone. The 10-year overall survival was 93% (95% CI 86%-96%). DISCUSSION: This study showed current epidemiologic and clinical status of POEMS syndrome in Japan. A quarter of patients were still inadequately treated with corticosteroid alone, whereas either autologous stem cell transplantation or immunomodulatory drugs improved the prognosis.

Original languageEnglish
Pages (from-to)e975-e983
JournalNeurology
Volume93
Issue number10
DOIs
Publication statusPublished - 2019 Sep 3

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POEMS Syndrome
Stem Cell Transplantation
Adrenal Cortex Hormones
Thalidomide
Skin
Japan
Confidence Intervals
Epidemiologic Methods
Melphalan
Polyneuropathies
Hematology
Neurology
Plasma Cells
Surveys and Questionnaires
Age of Onset
Vascular Endothelial Growth Factor A
Edema
Proteins
Cell Proliferation
Radiation

ASJC Scopus subject areas

  • Clinical Neurology

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Suichi, T., Misawa, S., Beppu, M., Takahashi, S., Sekiguchi, Y., Shibuya, K., ... Kuwabara, S. (2019). Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey. Neurology, 93(10), e975-e983. https://doi.org/10.1212/WNL.0000000000008062

Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey. / Suichi, Tomoki; Misawa, Sonoko; Beppu, Minako; Takahashi, Sho; Sekiguchi, Yukari; Shibuya, Kazumoto; Amino, Hiroshi; Tsuneyama, Atsuko; Suzuki, Yo Ichi; Nakamura, Keigo; Sato, Yasunori; Kuwabara, Satoshi.

In: Neurology, Vol. 93, No. 10, 03.09.2019, p. e975-e983.

Research output: Contribution to journalArticle

Suichi, T, Misawa, S, Beppu, M, Takahashi, S, Sekiguchi, Y, Shibuya, K, Amino, H, Tsuneyama, A, Suzuki, YI, Nakamura, K, Sato, Y & Kuwabara, S 2019, 'Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey', Neurology, vol. 93, no. 10, pp. e975-e983. https://doi.org/10.1212/WNL.0000000000008062
Suichi T, Misawa S, Beppu M, Takahashi S, Sekiguchi Y, Shibuya K et al. Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey. Neurology. 2019 Sep 3;93(10):e975-e983. https://doi.org/10.1212/WNL.0000000000008062
Suichi, Tomoki ; Misawa, Sonoko ; Beppu, Minako ; Takahashi, Sho ; Sekiguchi, Yukari ; Shibuya, Kazumoto ; Amino, Hiroshi ; Tsuneyama, Atsuko ; Suzuki, Yo Ichi ; Nakamura, Keigo ; Sato, Yasunori ; Kuwabara, Satoshi. / Prevalence, clinical profiles, and prognosis of POEMS syndrome in Japanese nationwide survey. In: Neurology. 2019 ; Vol. 93, No. 10. pp. e975-e983.
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abstract = "OBJECTIVE: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. METHODS: We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. RESULTS: The estimated number of patients with POEMS was 392 (95{\%} confidence interval [CI] 320-464), and the prevalence was 0.3 per 100,000. Detailed clinical profiles were available for 167 patients. Median age at onset was 54 years (range, 21-84 years), and the ratio of male to female was 1.5. All patients showed polyneuropathy; 89{\%} had monoclonal plasma cell proliferation; and 84{\%} had elevated vascular endothelial growth factor level in whom pretreatment serum or plasma was available (n = 87). Other common features were skin changes (84{\%}), edema/effusion (81{\%}), and organomegaly (76{\%}). A total of 160 patients were treated with any of the following: radiation, corticosteroids, melphalan, thalidomide, lenalidomide, bortezomib, or autologous stem cell transplantation. Primary therapeutic options were thalidomide (n = 86) and autologous stem cell transplantation (n = 71). Thirty-nine patients (24{\%}) were initially treated with corticosteroid alone. The 10-year overall survival was 93{\%} (95{\%} CI 86{\%}-96{\%}). DISCUSSION: This study showed current epidemiologic and clinical status of POEMS syndrome in Japan. A quarter of patients were still inadequately treated with corticosteroid alone, whereas either autologous stem cell transplantation or immunomodulatory drugs improved the prognosis.",
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AU - Sekiguchi, Yukari

AU - Shibuya, Kazumoto

AU - Amino, Hiroshi

AU - Tsuneyama, Atsuko

AU - Suzuki, Yo Ichi

AU - Nakamura, Keigo

AU - Sato, Yasunori

AU - Kuwabara, Satoshi

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N2 - OBJECTIVE: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. METHODS: We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. RESULTS: The estimated number of patients with POEMS was 392 (95% confidence interval [CI] 320-464), and the prevalence was 0.3 per 100,000. Detailed clinical profiles were available for 167 patients. Median age at onset was 54 years (range, 21-84 years), and the ratio of male to female was 1.5. All patients showed polyneuropathy; 89% had monoclonal plasma cell proliferation; and 84% had elevated vascular endothelial growth factor level in whom pretreatment serum or plasma was available (n = 87). Other common features were skin changes (84%), edema/effusion (81%), and organomegaly (76%). A total of 160 patients were treated with any of the following: radiation, corticosteroids, melphalan, thalidomide, lenalidomide, bortezomib, or autologous stem cell transplantation. Primary therapeutic options were thalidomide (n = 86) and autologous stem cell transplantation (n = 71). Thirty-nine patients (24%) were initially treated with corticosteroid alone. The 10-year overall survival was 93% (95% CI 86%-96%). DISCUSSION: This study showed current epidemiologic and clinical status of POEMS syndrome in Japan. A quarter of patients were still inadequately treated with corticosteroid alone, whereas either autologous stem cell transplantation or immunomodulatory drugs improved the prognosis.

AB - OBJECTIVE: To elucidate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. METHODS: We conducted a nationwide survey in 2015 using an established epidemiologic method. Data processing sheets were sent to all neurology and hematology specialist departments throughout Japan to identify patients with POEMS who were seen between April 2012 and March 2015. RESULTS: The estimated number of patients with POEMS was 392 (95% confidence interval [CI] 320-464), and the prevalence was 0.3 per 100,000. Detailed clinical profiles were available for 167 patients. Median age at onset was 54 years (range, 21-84 years), and the ratio of male to female was 1.5. All patients showed polyneuropathy; 89% had monoclonal plasma cell proliferation; and 84% had elevated vascular endothelial growth factor level in whom pretreatment serum or plasma was available (n = 87). Other common features were skin changes (84%), edema/effusion (81%), and organomegaly (76%). A total of 160 patients were treated with any of the following: radiation, corticosteroids, melphalan, thalidomide, lenalidomide, bortezomib, or autologous stem cell transplantation. Primary therapeutic options were thalidomide (n = 86) and autologous stem cell transplantation (n = 71). Thirty-nine patients (24%) were initially treated with corticosteroid alone. The 10-year overall survival was 93% (95% CI 86%-96%). DISCUSSION: This study showed current epidemiologic and clinical status of POEMS syndrome in Japan. A quarter of patients were still inadequately treated with corticosteroid alone, whereas either autologous stem cell transplantation or immunomodulatory drugs improved the prognosis.

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