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Published on: February 19, 2021
POEMS syndrome: clinical update
Rachel Brown1,2, Lionel Ginsberg3,4
1Department of Neurology, Royal Free Hospital, Pond Street, London, NW3 2QG, UK.
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.
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.
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