title: New advances in the diagnosis and treatment of POEMS syndrome.
abstract: 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 5 years. 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.
authors: Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
authors: Jian
authors: J
authors: Li
authors: Dao-bin
authors: DB
authors: Zhou
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D000068258:Bevacizumab; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D005871:Castleman Disease / Q000209:etiology; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D019468:Disease Management; D004359:Drug Therapy, Combination; D005803:Genes, Immunoglobulin; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D000077269:Lenalidomide; D008558:Melphalan / Q000008:administration & dosage / Q000627:therapeutic use; D010026:Osteosclerosis / Q000209:etiology; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D010950:Plasma Cells / Q000473:pathology; D011379:Prognosis; D011719:Pyrazines / Q000627:therapeutic use; D012720:Severity of Illness Index; D063189:Symptom Assessment; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives / Q000627:therapeutic use; D014182:Transplantation, Autologous; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors / Q000097:blood / Q000502:physiology
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D001897:Boronic Acids; D011719:Pyrazines; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000068258:Bevacizumab; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D000077269:Lenalidomide; D008558:Melphalan