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 |