title: Clinical characteristics and long-term outcome of patients with POEMS syndrome in China.
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.
authors.affiliation: Department of Hematology, Peking Union Medical College Hospital, Beijing, 100730, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Li
authors.forename: Dao-Bin
authors.initials: DB
authors.lastname: Zhou
authors.forename: Zhen
authors.initials: Z
authors.lastname: Huang
authors.forename: Li
authors.initials: L
authors.lastname: Jiao
authors.forename: Ming-Hui
authors.initials: MH
authors.lastname: Duan
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.forename: Yong-Qiang
authors.initials: YQ
authors.lastname: Zhao
authors.forename: Ti
authors.initials: T
authors.lastname: Shen
mesh_terms: D000328:Adult; D000368:Aged; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use; D005871:Castleman Disease / Q000473:pathology / Q000503:physiopathology; D002681:China; D005260:Female; D006801:Humans; D007668:Kidney / Q000378:metabolism; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology* / Q000503:physiopathology; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D018906:Antineoplastic Agents, Alkylating; D008558:Melphalan