. 2012 Apr 30;119(24):5650–5658. doi: 10.1182/blood-2012-03-378992

Table 1.

Criteria for the diagnosis of POEMS syndrome

	Criteria/other symptoms and signs	Affected, %^*
Mandatory major criteria (both required)	1. Polyradiculoneuropathy (typically demyelinating)	100
	2. Monoclonal plasma cell disorder (almost always λ)	100^†
Other major criteria (1 required)	3. Castleman disease^‡	11-25
	4. Sclerotic bone lesions	27-97
	5. VEGF elevation^§
Minor criteria (1 required)	6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)	45-85
	7. Extravascular volume overload (edema, pleural effusion, or ascites)	29-87
	8. Endocrinopathy (adrenal, thyroid,^‖ pituitary, gonadal, parathyroid, pancreatic^‖)	67-84
	9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, white nails)	68-89
	10. Papilledema	29-64
	11. Thrombocytosis/polycythemia^¶	54-88
Other symptoms and signs	Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B₁₂ values

The diagnosis of POEMS syndrome is confirmed when both of the mandatory major criteria, 1 of the 3 other major criteria, and 1 of the 6 minor criteria are present.

Summary of frequencies of POEMS syndrome features based on largest retrospective series.^2,7–11

^†

Takasuki and Nakanishi series are included, even though only 75% of patients had a documented plasma cell disorder. Because these are among the earliest series describing the syndrome, they are included.

^‡

There is a Castleman disease variant of POEMS syndrome that occurs without evidence of a clonal plasma cell disorder that is not accounted for in this table. This entity should be considered separately.

^§

A plasma VEGF level of 200 pg/mL is 95% specific and 68% sensitive for a POEMS syndrome.²⁸

^‖

Because of the high prevalence of diabetes mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this minor criterion.

^¶

Approximately 50% of patients will have bone marrow changes that distinguish it from a typical monoclonal gammopathy of undetermined significance or myeloma bone marrow.³⁷