Criteria for the Diagnosis of POEMS Syndrome

POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder (PCD). The acronym, which was coined by Bardwick in 1980, refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal PCD, and skin changes.

Criteria for the Diagnosis of POEMS Syndrome

Mandatory major criteria
1. Polyneuropathy (typically demyelinating)
2. Monoclonal plasma cell‐proliferative disorder (almost always λ)

Other major criteria
3. Castleman disease*

One required
4. Sclerotic bone lesions
5. VEGF elevation

Minor criteria
6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)
7. Extravascular volume overload (edema, pleural effusion, or ascites)
8. Endocrinopathy (adrenal, thyroid**, pituitary, gonadal, parathyroid, and pancreatic**)
9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, and white nails)
10. Papilledema
11. Thrombocytosis/polycythemia***

Other symptoms and signs
Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B12 values

 

POEMS, polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes.
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.

* There is a Castleman disease variant of POEMS syndrome that occurs without evidence of a clonal PCD 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.

*** Approximately 50% of patients will have bone marrow changes that distinguish it from a typical monoclonal gammopathy of undetermined significance (MGUS) or myeloma bone marrow. Anemia and/or thrombocytopenia are distinctively unusual in this syndrome unless Castleman disease is present

 

References:

  1. Dispenzieri A. POEMS syndrome: 2017 Update on diagnosis, risk stratification, and management. Am J Hematol. 2017 Aug;92(8):814-829. [Medline]
  2. Dispenzieri A, Kourelis T, Buadi F. POEMS Syndrome: Diagnosis and Investigative Work-up. Hematol Oncol Clin North Am. 2018 Feb;32(1):119-139. [Medline]

 

Created Jul 11, 2018.

print