title: [Efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) on the neuropathic manifestations in POEMS syndrome].
abstract: BACKGROUND
  POEMS syndrome (polyneuropathy, organomegaly, endocrynopathy, M-protein, and skin changes) is a rare multisystem disease associated with plasma cell dyscrasia. The efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) reported in case series has been mainly based on hematologic criteria and clinical recovery of peripheral neuropathy dysfunctions but has not been specifically evaluated. This retrospective study aimed to analyze the efficacy of auto-PBSCT on disability and electrophysiological patterns in patients with POEMS syndrome.
  
  
  METHODS
  Five patients presenting with POEMS syndrome received auto-PBSCT. Disability was evaluated before treatment and at 6 and 12 months using the Overall Neuropathy Limitation Scale (ONLS) and MRC sumscore of 28 muscles. Nerve conduction studies were performed before and one year after treatment, on median, ulnar, fibular and tibial nerves.
  
  
  RESULTS
  Mean age was 60.6 years (49-70). Disease duration between first symptoms and auto-PBSCT was 15.4 months (2-33). Before auto-PBSCT, mean ONLS score was 4.2 (1-10) and mean MRC sumscore 115.8/140 (74-140). At M6, mean ONLS score decreased and mean MRC sumscore increased; both were improved in all patients at M12: mean ONLS score 3 (range 0-8) at M6 and 2.2 (range 0-7) at M12; mean MRC sumscore 118/140 (77-140) at M6 and 122.4/140 (80-140) at M12. Significant recovery in electrophysiological patterns was observed in all patients on ulnar and median nerves: before-after treatment differences were observed for motor conduction velocities (34.41 vs. 45.47 m/s; P<0.001), distal CMAP amplitudes (5.04 vs. 5.96 mV; P=0.004), and sensory conduction velocities (43.20 vs. 49.20 m/s; P=0.001). Distal CMAP amplitude remained low in fibular and tibial nerves (0.41 vs. 0.17 mV).
  
  
  CONCLUSIONS
  Clinical and electrophysiological improvement is obvious in POEMS syndrome peripheral neuropathy within one year after treatment with auto-PBSCT, undoubtedly resulting from extensive remyelinisation and axonal regeneration. Further studies are required to examine long-term outcome in patients with POEMS syndrome given auto-PBSCT.
authors.affiliation: Centre de référence maladies neuromusculaires et SLA, pôle neurosciences cliniques, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France. Electronic address: sophiegronier@hotmail.com.
authors.forename: S
authors.initials: S
authors.lastname: Gronier
authors.affiliation: Centre de référence maladies neuromusculaires et SLA, pôle neurosciences cliniques, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
authors.forename: E
authors.initials: E
authors.lastname: Delmont
authors.affiliation: Service d'hématologie, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
authors.forename: L
authors.initials: L
authors.lastname: Legros
authors.affiliation: Explorations fonctionnelles, pôle neurosciences cliniques, hôpital Pasteur, CHU de Nice, 30, avenue de la Voie Romaine, 06002 Nice cedex 1, France.
authors.forename: M
authors.initials: M
authors.lastname: Launay
authors.affiliation: Service de médecine interne, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
authors.forename: P Y
authors.initials: PY
authors.lastname: Jeandel
authors.affiliation: Service de médecine interne, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
authors.forename: J G
authors.initials: JG
authors.lastname: Fuzibet
authors.affiliation: Centre de référence maladies neuromusculaires et SLA, pôle neurosciences cliniques, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
authors.forename: C
authors.initials: C
authors.lastname: Desnuelle
mesh_terms: D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000150:complications / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D010523:Peripheral Nervous System Diseases / Q000209:etiology / Q000628:therapy*; D014182:Transplantation, Autologous; D016896:Treatment Outcome
keywords: Autogreffe de moelle; Autologous peripheral blood stem cell transplantation; Electromyography; Neuropathie périphérique; POEMS syndrome; Peripheral nervous system diseases; Syndrome POEMS; Électroneuromyographie