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Criteria for the diagnosis of POEMS syndrome

Criteria for the diagnosis of POEMS syndrome
Mandatory major criteria (both required)
Polyneuropathy
Monoclonal plasma cell proliferative disorder (almost always lambda)
Other major criteria (one required)
Sclerotic bone lesions
Castleman disease
Elevated levels of vascular endothelial growth factor (VEGF)*
Minor criteria (one required)
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 hemangiomata, plethora, acrocyanosis, flushing, white nails)
Papilledema
Thrombocytosis/polycythemia
The diagnosis of POEMS syndrome is confirmed when both of the mandatory major criteria, one of the three other major criteria, and one of the six minor criteria are present.
* The source data do not define an optimal cut off value for considering elevated VEGF level as a major criterion. We suggest that VEGF measured in the serum or plasma should be at least three- to fourfold higher than the normal reference range for the laboratory that is doing the testing to be considered a major criterion.
¶ In order to consider endocrinopathy as a minor criterion, an endocrine disorder other than diabetes or hypothyroidism is required since these two disorders are common in the general population.
Original figure modified for this publication. Dispenzieri A. POEMS syndrome. Blood Rev 2007; 21:285. Illustration used with the permission of Elsevier Inc. All rights reserved.
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