POEMS syndrome

Overview

Type of disease: Rare conditions

POEMS syndrome is a rare blood disorder. It is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma proliferative disorder, and Skin changes. Signs and symptoms may include progressive sensorimotor polyneuropathy, enlarged liver and spleen, swollen lymph nodes, and darkening of the skin. Further testing and evaluation may show plasma cell disorder and sclerotic bone lesions. Many people with POEMS syndrome have diabetes (20-50%) and primary gonadal failure (55-70%). Other general signs and symptoms may include increased body hair, thickening of the skin, whitening of the nails, fatigue, weakness, swelling of the ankles and legs, enlarged breast tissue, generalized aches and pains, fluid in the lungs, and vision changes. Sign and symptoms tend to begin in a person’s 40’s to 50’s. POEMS syndrome is a rapidly progressive disorder that can become life threatening. Currently the exact cause of POEMS syndrome is unknown.  Researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.

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