The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

Abstract

POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells. The name POEMS is an acronym for some of the major disease manifestations, namely polyneuropathy, organomegaly, endocrinopathy, presence of monoclonal component, and skin changes. The clinical presentation can be various and could lead to delayed diagnosis and treatment. Little is known about the pathogenic mechanism, although the neoangiogenesis due to overproduction of vascular endothelial growth factor (VEGF) by plasma cells seems to play a key role. The latest evidence suggests that the blood concentration of this cytokine correlates with the activity of the syndrome: VEGF could then be used as a therapeutic target and a marker to monitor response. Several case reports have shown the efficacy of this approach, but extended studies are required to better define the use of anti-VEGF in patients affected by POEMS syndrome.

Keywords

POEMS syndrome / VEGF / anti-VEGF / plasma cell disorder

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