Clonal plasma cell dyscrasia is considered causative of both POEMS syndrome and primary light chain amyloidosis (AL-A). Patients with POEMS syndrome and AL-A may share clinical features, among which polyneuropathy, making the differential diagnosis sometimes difficult particularly in the neurological setting. Since vascular endothelial growth factor (VEGF), likely produced by the clonal plasma cell, reaches very high serum level in patients with POEMS syndrome and is considered a hallmark of the disease, we analyzed serum VEGF levels in patients with AL-A. Seventeen patients with AL-A were considered. Median VEGF level was 420 pg/ml in AL-A patients, significantly lower than in POEMS syndrome (median 2580 pg/ml, p = 0.000012). Follow-up of AL-A patients showed no changes in VEGF with regard to therapy response. Serum VEGF levels were not increased in AL-A patients and did not mirror the course of the disease, therefore they may help in the differential diagnosis in cases with overlapping clinical features.

Serum vascular endothelial growth factor (VEGF) in the differential diagnosis of amyloid neuropathy and POEMS syndrome.

FABRIZI, Gian Maria;
2011

Abstract

Clonal plasma cell dyscrasia is considered causative of both POEMS syndrome and primary light chain amyloidosis (AL-A). Patients with POEMS syndrome and AL-A may share clinical features, among which polyneuropathy, making the differential diagnosis sometimes difficult particularly in the neurological setting. Since vascular endothelial growth factor (VEGF), likely produced by the clonal plasma cell, reaches very high serum level in patients with POEMS syndrome and is considered a hallmark of the disease, we analyzed serum VEGF levels in patients with AL-A. Seventeen patients with AL-A were considered. Median VEGF level was 420 pg/ml in AL-A patients, significantly lower than in POEMS syndrome (median 2580 pg/ml, p = 0.000012). Follow-up of AL-A patients showed no changes in VEGF with regard to therapy response. Serum VEGF levels were not increased in AL-A patients and did not mirror the course of the disease, therefore they may help in the differential diagnosis in cases with overlapping clinical features.
amyloidosis; neuropathy; diagnosis; POEMS syndrome
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11562/428741
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