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Table 1 Criteria for the diagnosis of POEMS syndrome

From: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report

Mandatory major criteria

Polyneuropathy

Monoclonal plasmaproliferative disorder

Other major criteria

Castleman’s disease

Sclerotic bone lesions

Elevated serum or plasma vascular endothelial growth factor (VEGF) levels

Minor criteria

Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)

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

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

Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangiomata, white nails)

Papilledema

Thrombocytosis or polycythemia

  1. The existence of both mandatory major criteria and at least one of the other major criteria and one of the minor criteria are necessary for the diagnosis [4].