Abstract Details

Title Efficacy of Autologous Peripheral Blood Stem Cell Transplantation (Auto-PBSCT) on the Neuropathic Manifestations in POEMS Syndrome

Topic Peripheral Nerve

Presentation(s) P01 - (-)

Poster/Presentation
Number 147

Objective

Background BACKGROUND: POEMS syndrome (Polyneuropathy, organomegaly, endocrynopathy, M-protein, and skin changes) is a rare multisystem disease associated with a plasma cell dyscrasia. Efficacy of Auto-PBSCT have been reported in case series on haematologic criteria only, clinical recovery of peripheral neuropathy dysfunctions was not specifically evaluated.

Design/Methods DESIGN/METHODS: Five patients presenting with POEMS syndrome received Auto-PBSCT. Disability was evaluated before treatment and at 6 and 12 months through 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, peroneal and tibial nerves. Mann-Whitney test was used for statistical analysis.

Results RESULTS: Mean age was 60.6 years (49-70yr). 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 MRC sumscore 115.8/140 (74-140). At M6, mean ONLS score was decreased and MRC sumscore was increased and both were improved in all patients at M12, respectively at M6 ONLS mean score 3 (range 0-8) MRC sumscore 118.4/140 (77-140), at M12 ONLS mean score 2.75 (range 0-7) and MRC sumscore 118/140 (80-140). Significant recovery in ENMG patterns nerve was observed in all patients on ulnar and median nerves including motor conduction velocities, distal CMAP amplitudes and sensory conduction velocities with respective change in between before treatment and M12 of 34.41 vs 45.47 m/s, (p<0.001), 5.04 vs 5.96 mV (p=0.004) and 43.20 vs 49.20 m/s (p=0.001). Distal CMAP amplitude remained low in peroneal and tibial nerves (0.41 vs 0.17 mV).

Conclusions CONCLUSIONS: Auto-PBSCT in POEMS syndrome results to obvious both clinical and electrophysiological improvement of peripheral neuropathy within one year after treatment, which could be assume through extensive remyelinisation and axonal regeneration.

Authors/Disclosures
Sophie Gronier PRESENTER	No disclosure on file
Emilien Delmont, MD	No disclosure on file
Giancarlo Comi, MD (University Vita-Salute)	Dr. Comi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bristol Myers Squibb. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Janssen. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bristol Myers Squibb. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Aspen Healthcare. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Sanofi. Dr. Comi has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Rewind.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Claude J. Desnuelle, MD (CHU De Nice - Hopital Pasteur2)	No disclosure on file

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