POEMS syndrome diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

According to the The International Myeloma Working Group, the diagnosis of POEMS syndrome may be established if both mandatory criteria, one major and one minor criteria are fulfilled. Mandatory criteria include presence of polyneuropathy and a monoclonal plasma-cell proliferative disorder. Major criteria include presence of sclerotic bone lesions or mixed sclerotic/lytic lesions, presence of Castleman's disease and elevated serum VEGF levels. Minor criteria include presence of organomegaly, extra-vascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis and polycythemia.

Diagnostic Criteria

According to the The International Myeloma Working Group, the diagnosis of POEMS syndrome may be established if both mandatory criteria, at least one major and at least one minor criteria are fulfilled. The following table enlists the mandatory, major and minor criteria required for the diagnosis of POEMS syndrome:

Mandatory Criteria	Major Criteria	Minor Criteria
Polyneuropathy A monoclonal plasma-cell proliferative disorder	Sclerotic bone lesions or mixed sclerotic/lytic lesions Castleman's disease Elevated VEGF levels	Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy) Extra-vascular volume overload (peripheral edema, ascites, pleural effusion) Endocrinopathy: Adrenal Thyroid Pituitary Gonadal Parathyroid Pancreatic disorders excluding diabetes mellitus and hypothyroidism Skin changes: Hyperpigmentation Hypertricosis Glomeruloid hemangiomata Phletora Acrocyanosis Flushing Leukonychia Papilledema Thrombocytosis Polycythemia