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POEMS syndrome: A multisystem clonal disorder.

Tayyaba Ali1, Muzaffar H Qazilbash2

  • 1Department of Medicine, Karachi Medical and Dental College, Karachi, Pakistan.

European Journal of Haematology
|September 5, 2020
PubMed
Summary

POEMS syndrome is a rare multisystem disorder characterized by peripheral neuropathy and a plasma cell disorder. Vascular Endothelial Growth Factor (VEGF) overproduction is a key factor in its pathophysiology and diagnosis.

Keywords:
Castleman diseasePOEMS syndromehematopoietic stem cell transplantationparaproteinemiapolyneuropathy

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Area of Science:

  • Hematology
  • Neurology
  • Endocrinology

Background:

  • POEMS syndrome is a rare multisystem disorder.
  • Its pathophysiology is not fully understood.
  • Vascular Endothelial Growth Factor (VEGF) overproduction is implicated.

Purpose of the Study:

  • To summarize the key features of POEMS syndrome.
  • To outline diagnostic criteria.
  • To review current treatment approaches.

Main Methods:

  • Review of existing literature on POEMS syndrome.
  • Analysis of diagnostic criteria.
  • Summary of treatment strategies based on disease extent.

Main Results:

  • POEMS syndrome diagnosis requires polyneuropathy, a monoclonal plasma cell disorder, and specific major/minor criteria.
  • Elevated VEGF levels are a major diagnostic criterion.
  • Treatment varies from radiation therapy for limited disease to chemotherapy and stem cell transplantation for widespread disease.

Conclusions:

  • POEMS syndrome is a complex disorder with a significant role for VEGF.
  • Diagnosis relies on a combination of clinical and laboratory findings.
  • Treatment is tailored to the extent of bone involvement.