POEMS or Crow-Fukase syndrome is a rare plasma cell disease with multiorgan involvement, whose acronym refers to the presence of Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes. The etiology of POEMS is still unknown, but increasing evidence points to a possible role of Vascular Endothelial Growth Factor (VEGF). Serum VEGF levels have repeatedly been found to correlate with the clinical course and response to therapy. Lacking specific tests or patognomonic signs, the diagnosis of POEMSsyndrome is still based on the combination of clinical findings,although general consensus has still to be reached. Additional diagnostic markers other than clinical are therefore warranted. We herein report a patient who, although presenting all the POEMS features, was eventually diagnosed with a different disease.

Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes: not always a POEMS syndrome.

BRIANI, CHIARA;ADAMI, FAUSTO
2006

Abstract

POEMS or Crow-Fukase syndrome is a rare plasma cell disease with multiorgan involvement, whose acronym refers to the presence of Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes. The etiology of POEMS is still unknown, but increasing evidence points to a possible role of Vascular Endothelial Growth Factor (VEGF). Serum VEGF levels have repeatedly been found to correlate with the clinical course and response to therapy. Lacking specific tests or patognomonic signs, the diagnosis of POEMSsyndrome is still based on the combination of clinical findings,although general consensus has still to be reached. Additional diagnostic markers other than clinical are therefore warranted. We herein report a patient who, although presenting all the POEMS features, was eventually diagnosed with a different disease.
2006
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/1560433
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