Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome.
BACKGROUND
Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment.


OBJECTIVE
To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS syndrome.


METHODS
Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA.


RESULTS
Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms.


CONCLUSIONS
Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome with younger onset even if they are tetraplegic.
Kuwabara
S
S
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan. kuwabara-s@faculty.chiba-u.jp
Misawa
S
S
Kanai
K
K
Suzuki
Y
Y
Kikkawa
Y
Y
Sawai
S
S
Hattori
T
T
Nishimura
M
M
Nakaseko
C
C
D000200:Action Potentials / Q000502:physiology; D000328:Adult; D003131:Combined Modality Therapy; D004358:Drug Therapy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D018737:Hand Strength / Q000502:physiology*; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology*; D016878:POEMS Syndrome / Q000097:blood / Q000503:physiopathology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation / Q000379:methods*; D012189:Retrospective Studies; D013997:Time Factors; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000097:blood
D042461:Vascular Endothelial Growth Factor A