Annals of Hematology

, Volume 90, Issue 7, pp 819–826

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

  • Jian Li
  • Dao-Bin Zhou
  • Zhen Huang
  • Li Jiao
  • Ming-Hui Duan
  • Wei Zhang
  • Yong-Qiang Zhao
  • Ti Shen
Original Article

DOI: 10.1007/s00277-010-1149-0

Cite this article as:
Li, J., Zhou, DB., Huang, Z. et al. Ann Hematol (2011) 90: 819. doi:10.1007/s00277-010-1149-0

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.

Keywords

POEMS syndromeCastleman’s diseaseLong-term outcomeNeuropathy

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Jian Li
    • 1
  • Dao-Bin Zhou
    • 1
  • Zhen Huang
    • 1
  • Li Jiao
    • 1
  • Ming-Hui Duan
    • 1
  • Wei Zhang
    • 1
  • Yong-Qiang Zhao
    • 1
  • Ti Shen
    • 1
  1. 1.Department of HematologyPeking Union Medical College HospitalBeijingChina