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The Participant-Reported Implementation Update and Score PRIUS: A Novel Method for Capturing Implementation-Related Data Over Time

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POEMS syndrome: clinical update.

Rachel Brown^1,2, Lionel Ginsberg^3,4

¹Department of Neurology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK.

Journal of Neurology

|December 1, 2018

Summary

POEMS syndrome, a rare plasma cell disorder, causes a painful demyelinating neuropathy and multi-system issues. While high VEGF levels aid diagnosis, targeting VEGF has shown disappointing results, indicating other mechanisms are at play.

Keywords:

Monoclonal gammopathy Neuropathy Paraproteinaemia Vascular endothelial growth factor (VEGF)

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Area of Science:

Neurology
Oncology
Immunology

Background:

POEMS syndrome is a rare paraneoplastic syndrome linked to plasma cell disorders.
It presents with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes.
The neurological hallmark is a subacute, disabling demyelinating neuropathy.

Purpose of the Study:

To review the clinical features of POEMS syndrome.
To discuss differential diagnoses for POEMS syndrome.
To outline current treatment options for POEMS syndrome.

Main Methods:

Literature review of clinical features, diagnosis, and treatment of POEMS syndrome.
Analysis of the role of vascular endothelial growth factor (VEGF) in POEMS syndrome.
Discussion of current understanding of POEMS syndrome pathogenesis.

Main Results:

POEMS syndrome diagnosis requires a specific neuropathy and other systemic features.
Elevated VEGF levels are useful for diagnosis and monitoring treatment response.
Despite high VEGF, treatments targeting it have yielded disappointing results.

Conclusions:

POEMS syndrome is a complex disorder with diverse clinical manifestations.
VEGF plays a role but does not solely drive the disease, suggesting other mechanisms are involved.
Further research into pathogenesis and novel therapeutic targets is warranted.