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New advances in the diagnosis and treatment of POEMS syndrome

期刊

BRITISH JOURNAL OF HAEMATOLOGY
卷 161, 期 3, 页码 303-315

出版社

WILEY
DOI: 10.1111/bjh.12236

关键词

POEMS syndrome; vascular endothelial growth factor; autologous peripheral blood stem cell transplantation; melphalan; lenalidomide

资金

  1. Capital Research Fund on Clinical Application [Z111107058811019]
  2. Capital Development Fund [2011-4001-03]
  3. Peking Union Medical College New Star

向作者/读者索取更多资源

POEMS syndrome is a clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. Herein, the diagnostic criteria and characteristic features are reviewed, focusing the role of characteristic features in early diagnoses. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function. Autologous transplantation has resulted in a high response rate and durable remission. Moreover, transplantation-related morbidity and mortality has been significantly reduced over the past 5years. Induction therapy before transplantation may improve the harvest of stem cells and decrease transplantation-related morbidity. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients or those with organ dysfunction. Novel agents may also offer benefits to patients with a poor performance status or renal dysfunction, and transform transplantation eligibility.

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