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September 03, 2019; 93 (10) Article

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
First published August 1, 2019, DOI: https://doi.org/10.1212/WNL.0000000000008062
Tomoki Suichi
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Sonoko Misawa
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Minako Beppu
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Sho Takahashi
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Yukari Sekiguchi
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Kazumoto Shibuya
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Hiroshi Amino
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Atsuko Tsuneyama
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Yo-ichi Suzuki
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Keigo Nakamura
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Yasunori Sato
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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Satoshi Kuwabara
From the Department of Neurology (T.S., S.M., M.B., Y. Sekiguchi, K.S., H.A., A.T., Y.-i.S., K.N., S.K.), Graduate School of Medicine, Chiba University; Clinical Research Support Center (S.T.), Jikei University School of Medicine; and Department of Preventive Medicine and Public Health (Y. Sato), School of Medicine, Keio University, Tokyo, Japan.
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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
Neurology Sep 2019, 93 (10) e975-e983; DOI: 10.1212/WNL.0000000000008062

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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.

Glossary

ASCT=
autologous stem cell transplantation;
CI=
confidence interval;
POEMS=
polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes;
VEGF=
vascular endothelial growth factor

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • Received January 8, 2019.
  • Accepted in final form April 9, 2019.
  • © 2019 American Academy of Neurology
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