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POEMS Syndrome

(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)

by Jennifer M. Barker, MD

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.

POEMS syndrome is probably caused by circulating immunoglobulins caused by a plasma cell dyscrasia (see also Plasma Cell Disorders). Circulating cytokines (IL-1-β, IL-6), vascular endothelial growth factor, and tumor necrosis factor-α are also increased.

Patients may have the following:

  • Hepatomegaly

  • Lymphadenopathy

  • Hypogonadism

  • Diabetes mellitus type 2

  • Primary hypothyroidism

  • Hyperparathyroidism

  • Adrenal insufficiency (Addison disease)

  • Excess production of monoclonal IgA and IgG due to plasmacytomas

  • Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis)

Other symptoms and signs may include edema, ascites, pleural effusion, papilledema, and fever.

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 consists of chemotherapy and radiation therapy followed by autologous hematopoietic stem cell transplantation. Five-year survival is about 60%.

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