Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome.

Abstract

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.

CONCLUSION:

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.

• Authors: S Kuwabara, S Misawa, K Kanai, Y Suzuki, Y Kikkawa, S Sawai, T Hattori, M Nishimura, C Nakaseko
• Journal: Neurology (Neurology) Vol. 71 Issue 21 Pg. 1691-5 (Nov 18 2008) ISSN: 1526-632X [Electronic] United States
• PMID: 18832140 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Vascular Endothelial Growth Factor A

Topics

• Action Potentials (physiology)
• Adult
• Combined Modality Therapy
• Drug Therapy (methods)
• Female
• Follow-Up Studies
• Hand Strength (physiology)
• Humans
• Male
• Median Nerve (physiopathology)
• Middle Aged
• Neural Conduction (physiology)
• POEMS Syndrome (blood, physiopathology, therapy)
• Peripheral Blood Stem Cell Transplantation (methods)
• Retrospective Studies
• Time Factors
• Transplantation, Autologous
• Treatment Outcome
• Vascular Endothelial Growth Factor A (blood)