Disease Information for POEMS Plasma cell syndrome: Definition

  • Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare multisystemic disease that occurs in the setting of a plasma cell dyscrasia; The pathophysiologic link between

    the constellation of symptoms and the underlying disease is not well understood, but the link may be related to changes in the levels of a cytokine or a growth factor;

    5 main features of the disease, namely, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes; No specific case definition for POEMS exists; however, most authors agree that patients with POEMS syndrome should have 3 or more of the 5 features; The pathophysiology of the disease is not known; however, elevated levels of cytokines and growth factors have been implicated; The polyneuropathy associated with POEMS syndrome is a bilateral symmetric disturbance; It involves both motor and sensory nerves, begins distally, and has a progressive proximal spread; Associated cranial or autonomic nerves are not involved; Both demyelination and axonal degeneration are noted; The liver, the lymph nodes, and the spleen are the organs most frequently involved; Enlargement of the lymph nodes and spleen is secondary to changes consistent with Castleman disease (giant angiofollicular hyperplasia, multicentric plasma cell variant) in most patients; Hepatomegaly is not

    associated with any defined histologic or pathophysiologic changes; Multiple endocrinopathies have been associated with POEMS syndrome, and most patients have more than 1 endocrine abnormality; Many of the abnormalities noted can be explained by elevations in estrogen levels; Impotence and gynecomastia are common among men; Amenorrhea is common among women; Diabetes mellitus and glucose intolerance are also noted in many patients; Other associated endocrinopathies include hypothyroidism,

    hyperprolactinemia, and hypoparathyroidism; POEMS syndrome is seen in the setting of a plasma cell dyscrasia;