title: Pulmonary morbidity improves after autologous stem cell transplantation in POEMS syndrome.
abstract: BACKGROUND
  POEMS syndrome is a plasma cell disorder manifested by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes. Pulmonary morbidity includes restriction, decreased diffusing capacity for carbon monoxide (DLCO), respiratory muscle weakness, abnormal imaging, and pulmonary hypertension. Autologous peripheral blood stem cell transplantation (aPBSCT) is an effective treatment for POEMS syndrome. It is unknown if aPBSCT improves pulmonary morbidity. We hypothesize pulmonary morbidity will improve following aPBSCT.
  
  
  METHODS
  Retrospective cohort study of POEMS syndrome aPBSCT recipients from 2000 to 2010. Demographic, pulmonary function test (PFT), echocardiogram, cytokine, and imaging data at baseline and after aPBSCT were abstracted. Pre- and post-transplant data were compared using Wilcoxon signed-rank and McNemar's tests.
  
  
  RESULTS
  53 patients met criteria. Median improvements in forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), and total lung capacity (TLC) after transplant were 180, 315 and 350 ml respectively (median follow-up of 1.1 years). DLCO, maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) improved by a median of 11, 12.5 and 10% predicted respectively. RVSP and chest imaging also improved. Vascular endothelial growth factor and IL-6 decreased by a median of 334 and 2 pg/ml respectively. All comparisons were statistically significant. Longitudinal data demonstrated stability in FEV1, MEP, and TLC and continued improvement in FVC, MIP and DLCO on subsequent PFTs (median follow-up 26.5 months).
  
  
  CONCLUSIONS
  Patients with POEMS syndrome treated with aPBSCT have significant improvement in PFTs, respiratory muscle strength, imaging, and post-transplant IL-6. The improvement in PFTs persists during long-term follow-up.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55902, USA. Electronic address: Chandrashekaran.Satish@mayo.edu.
authors.forename: Satish
authors.initials: S
authors.lastname: Chandrashekaran
authors.affiliation: Division of Hematology, Mayo Clinic, 200 First St. SW, Rochester, MN 55902, USA. Electronic address: Dispenzieri.Angela@mayo.edu.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Department of Biomedical Statistics and Informatics, Mayo Clinic, 200 First St. SW, Rochester, MN 55902, USA. Electronic address: cha@mayo.edu.
authors.forename: Stephen S
authors.initials: SS
authors.lastname: Cha
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St. SW, Rochester, MN 55902, USA. Electronic address: kennedy.cassie@mayo.edu.
authors.forename: Cassie C
authors.initials: CC
authors.lastname: Kennedy
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000628:therapy; D015850:Interleukin-6 / Q000097:blood; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation / Q000379:methods*; D011859:Radiography; D012129:Respiratory Function Tests / Q000379:methods; D012131:Respiratory Insufficiency / Q000503:physiopathology / Q000628:therapy; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D015850:Interleukin-6
keywords: Hypertension, pulmonary; POEMS syndrome; Respiratory function tests; Stem cell transplantation