Fitting the Pieces of the POEMS Syndrome Jigsaw Puzzle-Guillain-Barre Syndrome in a Patient with Refractory Ascites: Case Report


Shorbagi A., ÖNAL İ. K. , Onal E. D. , KILIÇKAP S. , BARIŞTA İ. , Kansu T., ...More

TURKIYE KLINIKLERI TIP BILIMLERI DERGISI, vol.30, no.1, pp.352-355, 2010 (Journal Indexed in SCI) identifier identifier

  • Publication Type: Article / Article
  • Volume: 30 Issue: 1
  • Publication Date: 2010
  • Doi Number: 10.5336/medsci.2008-8193
  • Title of Journal : TURKIYE KLINIKLERI TIP BILIMLERI DERGISI
  • Page Numbers: pp.352-355

Abstract

POEMS is an acronym for a rare and poorly understood syndrome associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes that usually occurs in the setting of a plasma cell dyscrasia. Elevations of cytokines including vascular endothelial growth factor (VEGF) have all been noted. Enlargement of the lymph nodes and spleen is secondary to changes consistent with Castleman's disease (giant angiofollicular hyperplasia, multicentric plasma cell variant). There is no particular test that establishes the diagnosis of POEMS syndrome with certainty, and a diagnosis is usually made when seemingly unrelated signs and symptoms are linked. We present a case of a 39-year-old man who presented with massive ascites and organomegaly who developed Guillain-Barre syndrome-like neuropathy. He was eventually diagnosed with the POEMS syndrome. The syndrome should be kept in mind in the patients with refractory ascites and compatible clinical picture.