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Clinical characteristics and long-term outcome of patients with POEMS syndrome in China.
MedLine Citation:
PMID:  21221584     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Jian Li; Dao-Bin Zhou; Zhen Huang; Li Jiao; Ming-Hui Duan; Wei Zhang; Yong-Qiang Zhao; Ti Shen
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-8
Journal Detail:
Title:  Annals of hematology     Volume:  -     ISSN:  1432-0584     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107334     Medline TA:  Ann Hematol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Hematology, Peking Union Medical College Hospital, Beijing, 100730, China.
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