Table 1

Diagnostic criteria.

Mandatory major criteria	Polyneuropathy
Mandatory major criteria	Monoclonal plasma cell proliferative disorder (almost always l)
Any one of the following three other major criteria:	Sclerotic bone lesions
	Castleman’s disease
	Elevated levels of VEGF
Any one of the following six minor criteria:	Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)
	Extravascular volume overload (edema, pleural effusion, or ascites)
	Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
	Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangioma, plethora, acrocyanosis, flushing, white nails)
	Papilledema
	Thrombocytosis/polycythemia