POEMS Syndrome
(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)
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, and tumor necrosis factor-alpha are also increased.
Patients may have the following:
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Hepatomegaly
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Hypogonadism
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Diabetes mellitus type 2
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Primary hypothyroidism
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Adrenal insufficiency (Addison disease)
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Excess production of monoclonal IgA and IgG due to plasmacytomas
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Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis)
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Osteoclastic bone lesions
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Monoclonal gammopathy
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).
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms and signs. Criteria include the presence of polyneuropathy and monoclonal paraproteinemia plus any 2 of the other manifestations of the disorder.
Treatment
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
