Criteria for the diagnosis of POEMS syndrome*

Mandatory major criteria	Polyneuropathy (typically demyelinating) Monoclonal plasma cell-proliferative disorder (almost always lambda)
Other major criteria (one required)	Castleman disease* Sclerotic bone lesions Vascular endothelial growth factor elevation
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 hemangiomata, plethora, acrocyanosis, flushing, white nails) Papilledema Thrombocytosis/polycythemia^Δ
Other symptoms and signs	Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B₁₂ values

NOTE: 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.

POEMS: Polyneuropathy, Organomegaly, Endocrinopathy, M protein, Skin changes.

* 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.

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

Δ Approximately 50% of patients will have bone marrow changes that distinguish it from a typical MGUS or myeloma bone marrow.^[1] Anemia and/or thrombocytopenia are distinctively unusual in this syndrome unless Castleman disease is present.

Reference:

Briani C, Fabrizi GM, Ruggero S, et al. Vascular endothelial growth factor helps differentiate neuropathies in rare plasma cell dyscrasias. Muscle Nerve 2011; 43:164.

From: Dispenzieri A. POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management. Am J Hematol 2021; 96:872. https://onlinelibrary.wiley.com/doi/10.1002/ajh.26240. Copyright © 2021 Wiley Periodicals, LLC. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).

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Criteria for the diagnosis of POEMS syndrome*