Table 1 Diagnostic criteria for POEMS syndrome: [1]

Requires two mandatory criteria PLUS ≥ 1 major AND ≥ 1 minor criteria

Mandatory criteria

  Polyneuropathy (typically demyelinating)

  Monoclonal plasma cell-proliferative disorder (almost always λ)

Major criteria

  Castleman disease(lymphoproliferative disorder)

  Sclerotic bone lesions

  Vascular endothelial growth factor (VEGF) elevation

Minor criteria

  Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)

  Extravascular volume overload (edema, pleural effusion, or ascites)

  Endocrinopathy (adrenal, pituitary, gonadal, parathyroid, thyroid and pancreatic)

  Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, and white nails)

  Papilledema

  Thrombocytosis/polycythemia