Crow-Fukase syndrome

FREE subscriptions for doctors and students... click here
You have 3 more open access pages.

POEMS is an acronym for:

  • polyneuropathy:
    • slow onset of mixed motor and sensory polyneuropathy
    • peripheral nerve demyelination with some axonal degeneration
  • organomegaly:
    • hepatosplenomegaly
  • endocrinopathy:
    • gynaecomastia
    • secondary amenorrhoea
    • diabetes mellitus
    • hypothyroidism
  • monoclonal protein
  • skin changes:
    • hyperpigmentation
    • hypertrichosis
    • skin thickening

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell disorder characterized by demyelinating peripheral neuropathy and clonal plasma cell proliferation

  • clinical manifestations are believed to be associated with a surge of inflammatory and angiogenic mediators, including interleukins and vascular endothelial growth factor (VEGF), elicited by clonal and polyclonal plasma cells
  • underlying pathology is usually an osteosclerotic myeloma. Radiographic surveys often reveal a single osteosclerotic lesion in the spine or proximal long bones

POEMS syndrome may be underdiagnosed because of its rarity, and it can be mistaken for chronic inflammatory demyelinating polyneuropathy

  • therefore, in addition to measurement of the VEGF level, patients with a monoclonal protein detected in blood and/or urine and neuropathy should be evaluated for POEMS syndrome with use of imaging to assess whether sclerotic bone lesions, effusions, and organomegaly are present

Treatment is largely based on small case series in which plasma cell-directed therapies were used

  • high-dose melphalan and autologous hematopoietic cell transplantation may be offered to eligible patients (1)
  • lenalidomide and dexamethasone can be prescribed for patients who are ineligible for transplants (1)
  • main goals of therapy are to attain complete hematologic and VEGF responses and to reduce symptoms, although it may take up to 3 years for neurologic deficits to be ameliorated

Reference:

  • Khouri J, Nakashima M, Wong S. Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome: A Review. JAMA Oncol. 2021 Jun 3

Last edited 06/2021 and last reviewed 06/2021

Links: