title: Free light chains and heavy/light chains in monitoring POEMS patients.
abstract: BACKGROUND
  POEMS syndrome is defined by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes. The vascular endothelial growth factor (VEGF) appears to play a key role in the pathogenesis of the syndrome, and its concentrations are deemed to correlate to disease activity. The aim of the present study was to verify whether other biochemical markers including serum free light chains (FLC) and heavy/light chains (HLC) would be of value in monitoring POEMS patients.
  
  
  METHODS
  Fifty-three serum samples were collected from seven POEMS patients at diagnosis and during a follow-up period (range 14-56 months). VEGF was measured using an ELISA method, while FLC and HLC concentrations were measured using Binding Site reagents on a BNII (Siemens) nephelometer.
  
  
  RESULTS
  At diagnosis all patients presented high VEGF concentrations, while the κ/λFLC ratio (FLCr) was within the reference range. Four patients had abnormal HLC, HLCκ/HLCλ (HLCr) and FLC values. The relationship between the trend of VEGF and both HLC and FLC during the follow-up was analysed by means of Cohen's κ coefficient. VEGF and HLC values displayed a significant κ-Cohen (0.537, p=0.002) in all chemotherapy-responder patients while in non-responders it did not. Conversely, in both responders and non-responders, VEGF and FLC values did not attain a significance on κ-Cohen analysis. In three out of four responders HLCr values increased, thus reflecting an improved clinical condition.
  
  
  CONCLUSIONS
  The findings made in the present study indicate that HLC, either as intact immunoglobulin or as HLCr, may provide useful information, particularly in identifying responders and confirm that the role of FLC is unreliable in monitoring patients with POEMS syndrome.
authors.forename: Sara
authors.initials: S
authors.lastname: Altinier
authors.forename: Kozeta
authors.initials: K
authors.lastname: Proko
authors.forename: Martina
authors.initials: M
authors.lastname: Zaninotto
authors.forename: Daniela
authors.initials: D
authors.lastname: Ciubotaru
authors.forename: Mara
authors.initials: M
authors.lastname: Seguso
authors.forename: Mariacristina
authors.initials: M
authors.lastname: Varagnolo
authors.forename: Federica
authors.initials: F
authors.lastname: Lessi
authors.forename: Chiara
authors.initials: C
authors.lastname: Briani
authors.forename: Fausto
authors.initials: F
authors.lastname: Adami
authors.forename: Mario
authors.identifier: http://orcid.org/0000-0002-0270-1711
authors.initials: M
authors.lastname: Plebani
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000097:blood*; D007147:Immunoglobulin Light Chains / Q000097:blood*; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000276:immunology; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
chemical_list: D007143:Immunoglobulin Heavy Chains; D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D042461:Vascular Endothelial Growth Factor A