Criteria for the diagnosis of POEMS syndrome: diagnosis is confirmed when both mandatory major criteria, 1 of the 3 major criteria, and 1 of the 6 minor criteria are present
| Mandatory major criteria |
| • Polyneuropathy (typically demyelinating) • Monoclonal plasma cell–proliferative disorder (almost always lambda) |
| Major criteria |
| • Castleman disease • Sclerotic bone lesions • Increased levels of VEGF |
| Minor criteria |
| • Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) • Extravascular volume overload (edema, pleural effusion, or ascites) • Endocrinopathy (adrenal, thyroid,a pituitary, gonadal, parathyroid, pancreatica) • Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails) • Papilledema • Thrombocytosis or polycythemia |
| Other known associations |
| Clubbing, weight loss, hyperhidrosis, pulmonary hypertension or restrictive lung disease, thrombotic diathesis, diarrhea, low vitamin B12 values |
| Mandatory major criteria |
| • Polyneuropathy (typically demyelinating) • Monoclonal plasma cell–proliferative disorder (almost always lambda) |
| Major criteria |
| • Castleman disease • Sclerotic bone lesions • Increased levels of VEGF |
| Minor criteria |
| • Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) • Extravascular volume overload (edema, pleural effusion, or ascites) • Endocrinopathy (adrenal, thyroid,a pituitary, gonadal, parathyroid, pancreatica) • Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails) • Papilledema • Thrombocytosis or polycythemia |
| Other known associations |
| Clubbing, weight loss, hyperhidrosis, pulmonary hypertension or restrictive lung disease, thrombotic diathesis, diarrhea, low vitamin B12 values |
Diabetes mellitus and thyroid abnormalities alone are not sufficient to meet the minor criterion due to their high prevalence.
Adapted with permission from Dispenzieri.32