POEMS syndrome diagnostic criteria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Diagnostic Criteria
A 2003 study[1] followed a series of 99 patients and attempted to delineate criteria for the diagnosis of POEMS syndrome. According to their study, two major criteria and one minor criterion are required for the diagnosis:
- Major criteria:
- Polyneuropathy
- Monoclonal plasmaproliferative disorder (a paraprotein)
- Minor criteria:
- Sclerotic bone lesions
- Castleman disease
- Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy)
- Edema (edema, pleural effusion or ascites)
- Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, pancreatic)
- Skin changes (hyperpigmentation, hypertrichosis, plethora, hemangioma, white nails)
- Papilledema
In addition, their study showed a number of associations, the relevance of which to diagnosis was unclear: clubbing, weight loss, thrombocytosis, polycythemia and hyperhidrosis. Possible associations included pulmonary hypertension, restrictive lung disease, a thrombotic diathesis, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.