POEMS Syndrome: an Enigma

Curr Hematol Malig Rep. 2017 Apr;12(2):85-95. doi: 10.1007/s11899-017-0367-0.

Abstract

POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis. Treatment is based on extent of the disease. Radiotherapy is used for localized disease. Systemic therapy is required for disseminated disease, with bone marrow involvement by clonal plasma cells, or in patients who progress shortly after radiation. Upfront autologous stem cell transplantation is the treatment of choice for transplant-eligible patients. Outcomes are typically superior to that of standard myeloma. Herein, using a case vignette, we outline the latest evidence regarding the prognostication and management of POEMS syndrome, with a focus on its relapsing-remitting course.

Keywords: Immunomodulatory drugs; Monoclonal gammopathy; Neuropathy; Osteosclerotic myeloma; Paraneoplastic; Vascular endothelial growth factor.

Publication types

  • Review

MeSH terms

  • Biomarkers
  • Castleman Disease / diagnosis
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Humans
  • POEMS Syndrome / diagnosis*
  • POEMS Syndrome / etiology*
  • POEMS Syndrome / mortality
  • POEMS Syndrome / therapy*
  • Phenotype
  • Prognosis
  • Treatment Outcome

Substances

  • Biomarkers