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

Neurology® Sep 06, 2019

Suichi T, Misawa S, Beppu M, et al. - Using an established epidemiologic method, researchers carried out a nationwide survey in 2015 to illustrate current epidemiological, clinical profiles, and treatment of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome. In order to identify patients with POEMS who were seen between April 2012 and March 2015, data processing sheets were sent to all departments of neurology and hematology specialists across Japan. Data reported that the estimated number of POEMS patients was 392, with 0.3 prevalence per 100,000 patients. Polyneuropathy was shown by all patients. Skin changes, edema/effusion, and organomegaly were other common features. It was noted that the 10-year overall survival was 93%. A quarter of patients were still inadequately treated with corticosteroid alone, while the prognosis was improved either by autologous stem cell transplantation or immunomodulatory medications.
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