[Papilloedema and POEMS syndrome].

Oftalmologia

Clinica de Oftalmologie, Timişoara.

Published: October 2002

AI Article Synopsis

  • POEMS syndrome is characterized by five key features: polyneuropathy, organomegaly, endocrinopathy, monoclonal component, and skin changes, with chronic bilateral papilloedema occurring in many cases.
  • It is a multisystem disorder linked to plasma cell dyscrasia and often associated with Castleman disease, with factors like inflammatory cytokines and human herpes virus-8 suggested as contributors to its development.
  • The text discusses the case of a 16-year-old female patient diagnosed with POEMS syndrome, focusing on the symptoms over time and exploring the diagnosis, pathogenesis, and treatment options based on recent literature.

Article Abstract

POEMS syndrome stands for the first letter of the words: polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal component (M), changes of the tegument (S-skin). In 33-84% of the cases, chronic bilateral papilloedema is associated with the syndrome. The disease is a multisystem disorder, a plasma cell dyscrasia being present, often associated with Castleman disease. Inflammatory cytokines, endothelial vascular growth factor and human herpes virus-8 are thought to play an important role in pathogenesis. This summary is about a 16 year old female patient, where the diagnosis of POEMS syndrome was elaborated based on the systematization of the clinical symptomatology, developed over a long period of time. Based on the latest data from literature, peculiarities of diagnosis, pathogenesis and therapy of the disease are being discussed.

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