POEMS syndrome diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Diagnostic Criteria

The most recent criteria for the diagnosis of POEMS syndrome require that all of the following be present:

• Polyneuropathy
• A monoclonal plasma-cell proliferative disorder

• One or more of the following major criteria:
  • Sclerotic bone lesions
  • Castleman's disease
  • Elevated VEGF levels

• One or more of the following minor criteria:
  • Organomegaly (enlarged spleen, liver or lymph nodes)
  • Extravascular volume overload (oedema, pleural effusion or ascites)
  • Endocrinopathy (adrenal, pituitary, gonadal, parathyroid); diabetes or hypothyroidism alone are insufficient
  • Skin changes
  • Papilloedema
  • Thrombocytosis or polycythaemia

In addition, clubbing, weight loss, and hyperhidrosis can also be seen. Possible associations included pulmonary hypertension, restrictive lung disease, a thrombotic diathesis, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.