Last updated: November 3, 2014
Definition: A rare multisystem disorder associated with plasma cell dyscrasias.
Pathogenesis: Pathogenesis is unknown. Elevated serum concentrations of the proinflammatory cytokines IL-1, IL-6, and TNF-a and of the metalloproteinases TIMP and VEGF have been reported.
Cardinal Findings: The acronym POEMS represents the key features, including
—Plasma cell dyscrasia with polyneuropathy: Plasmocytomas often present as sclerotic bony lesions. A severe, sensorimotor inflammatory polyneuropathy is uncommon.
—Organomegaly: Hepatomegaly and lymphadenopathy are present in 67%; splenomegaly is seen in 33% of patients.
—Endocrinopathy: Hypothyroidism, hypogonadism with gynecomastia, amenorrhea, impotence, or hyperprolactinemia may be seen.
—Monoclonal gammopathy: IgG or IgA monoclonal paraproteinemia may be seen.
—Skin changes: Scleroderma-like skin changes are common, but changes may also include hyperpigmentation, hypertrichosis, hyperhidrosis, and angiomas.
—Other features: Fever, anorexia, and thrombocytosis occur. Rare associations include Castleman disease or vasculitis.
Therapy: Initially, most patients are treated with corticosteroids. Cyclophosphamide and other immunomodulatory agents have been used in refractory cases.