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[Efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) on the neuropathic manifestations in POEMS syndrome]. / Efficacité à moyen terme de l'autogreffe de cellules souches hématopoïétiques périphériques sur la polyneuropathie du syndrome POEMS: expérience chez 5 cas.
Gronier, S; Delmont, E; Legros, L; Launay, M; Jeandel, P Y; Fuzibet, J G; Desnuelle, C.
Affiliation
  • Gronier S; 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.
  • Delmont E; 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.
  • Legros L; Service d'hématologie, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
  • Launay M; Explorations fonctionnelles, pôle neurosciences cliniques, hôpital Pasteur, CHU de Nice, 30, avenue de la Voie Romaine, 06002 Nice cedex 1, France.
  • Jeandel PY; Service de médecine interne, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
  • Fuzibet JG; Service de médecine interne, hôpital l'Archet, CHU de Nice, 151, route Saint-Antoine-Ginestiere, 06202 Nice cedex 3, France.
  • Desnuelle C; 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.
Rev Neurol (Paris) ; 170(1): 37-45, 2014 Jan.
Article in Fr | MEDLINE | ID: mdl-24411685
INTRODUCTION: 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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: POEMS Syndrome / Peripheral Nervous System Diseases / Peripheral Blood Stem Cell Transplantation Type of study: Etiology_studies / Observational_studies / Prognostic_studies Limits: Aged / Female / Humans / Male / Middle aged Language: Fr Journal: Rev Neurol (Paris) Year: 2014 Document type: Article Country of publication: France

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: POEMS Syndrome / Peripheral Nervous System Diseases / Peripheral Blood Stem Cell Transplantation Type of study: Etiology_studies / Observational_studies / Prognostic_studies Limits: Aged / Female / Humans / Male / Middle aged Language: Fr Journal: Rev Neurol (Paris) Year: 2014 Document type: Article Country of publication: France