POEMS syndrome is probably caused by circulating immunoglobulins caused by a plasma cell disorder. Circulating cytokines (interleukin 1-beta, interleukin 6), vascular endothelial growth factor (VEGF), and tumor necrosis factor-alpha are also increased.
Symptoms and Signs
Patients may have the following:
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Adrenal insufficiency (Addison disease)
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Diabetes mellitus type 2
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Hepatomegaly
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Hypogonadism
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Monoclonal gammopathy
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Osteoclastic bone lesions
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Polyneuropathy (peripheral motor and sensory manifestations)
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Primary hypothyroidism
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Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis)
Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. About 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).
Diagnosis
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms, signs and laboratory findings.
Patients must have both mandatory criteria and ≥ 1 major and ≥ 1 minor criteria.
Mandatory criteria (both):
Major criteria (≥ 1):
Minor criteria (≥ 1):
Treatment of POEMS Syndrome
Treatment of POEMS syndrome consists of radiation therapy or chemotherapy for bony lesions or bone marrow involvement sometimes followed by autologous hematopoietic stem cell transplantation (1). Five-year survival is about 60%.
Treatment reference
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1. Dispenzieri A: POEMS syndrome: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 92:814–829, 2017. doi: 10.1002/ajh.24802
