POEMS Syndrome: Diagnosis and Investigative Work-up

Hematol Oncol Clin North Am. 2018 Feb;32(1):119-139. doi: 10.1016/j.hoc.2017.09.010.

Abstract

POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease is the first step in managing the disease. Increased blood levels of vascular endothelial growth factor are usually confirmatory. This rare disorder should not be missed, especially if the patient has a putative diagnosis of chronic inflammatory polyradiculoneuropathy, a lambda restricted monoclonal gammopathy, and thrombocytosis, and is not responding as expected to immunomodulatory therapy commonly used for chronic inflammatory polyradiculoneuropathy.

Keywords: Castleman disease; Chronic inflammatory polyradiculoneuropathy; Paraneoplastic; Plasma cell disorder.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunologic Factors / therapeutic use*
  • POEMS Syndrome* / blood
  • POEMS Syndrome* / diagnosis
  • POEMS Syndrome* / drug therapy
  • POEMS Syndrome* / pathology
  • Plasma Cells / metabolism
  • Plasma Cells / pathology
  • Vascular Endothelial Growth Factor A / blood

Substances

  • Immunologic Factors
  • Vascular Endothelial Growth Factor A