POEMS syndrome

Last revised by Chandrajit Prasad on 17 Jan 2024

POEMS syndrome is the acronymic name for a rare multisystem paraneoplastic disorder including the following features:

The diagnostic criteria differ from the acronym. According to the International Myeloma Working Group, the diagnosis of POEMS requires two mandatory criteria, one major criterion, and one minor criterion 3.

Mandatory criteria:

  • polyneuropathy

  • monoclonal plasma cell proliferative disorder (the light chain is almost always lambda)

Major criteria:

Minor criteria:

  • organomegaly (hepatomegalysplenomegaly, or lymphadenopathy)

  • extravascular volume overload (edema, pleural effusion, or ascites)

  • endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, or pancreatic)

  • skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, or white nails)

  • optic disc swelling

  • thrombocytosis/polycythemia

To make the diagnosis of POEMS, these features should be temporally related and have no other attributable cause.

Brain MRI discloses smooth, diffuse pachymeningeal thickening in most patients, sometimes with subdural effusions 4

Spine MRI shows thickening and enhancement of nerve roots in the branchial and lumbosacral plexi and occasionally also the intradural cauda equina nerve roots 4.

The bones show sclerotic lesions, usually small (<1 cm) and multiple 5. The larger lesions can have a radiolucent center and sclerotic margins 5.

Patients with POEMS are often initially misdiagnosed with chronic inflammatory demyelinating polyneuropathy 4.

ADVERTISEMENT: Supporters see fewer/no ads