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Erschienen in: Annals of Hematology 7/2011

01.07.2011 | Original Article

Clinical characteristics and long-term outcome of patients with POEMS syndrome in China

verfasst von: Jian Li, Dao-Bin Zhou, Zhen Huang, Li Jiao, Ming-Hui Duan, Wei Zhang, Yong-Qiang Zhao, Ti Shen

Erschienen in: Annals of Hematology | Ausgabe 7/2011

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Abstract

POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. This study reviewed the clinical characteristics and long-term outcome of 99 consecutive Chinese patients with newly diagnosed POEMS syndrome in a single institute. The median age of 99 patients was 45 years, and the ratio of men/women was 1.4. The median time from onset of symptoms to diagnosis was 18 months. The typical five features of peripheral neuropathy, organomegaly, endocrinopathy, M protein, and skin change remained to be essential for patients with POEMS syndrome in China. The unusual features like pulmonary hypertension (36%) and renal impairment (37%) were not uncommon in China. Eighty-three percent patients were alive after follow-up time of 25 months, and 10% patients had survived more than 60 months. Melphalan-based therapy (OR, 0.076; 95% CI, 0.02–0.285) and normal renal function (OR, 0.246; 95% CI, 0.076–0.802) were independent prognostic factors for the survival of patients with POEMS syndrome. In conclusion, POEMS syndrome in Chinese patients was a multi-systemic disease with clinical features similar to non-Chinese ones. Active therapy can effectively improve the prognosis of patients with POEMS syndrome.
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Metadaten
Titel
Clinical characteristics and long-term outcome of patients with POEMS syndrome in China
verfasst von
Jian Li
Dao-Bin Zhou
Zhen Huang
Li Jiao
Ming-Hui Duan
Wei Zhang
Yong-Qiang Zhao
Ti Shen
Publikationsdatum
01.07.2011
Verlag
Springer-Verlag
Erschienen in
Annals of Hematology / Ausgabe 7/2011
Print ISSN: 0939-5555
Elektronische ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-010-1149-0

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