National Organization for Rare Disorders, Inc.
It is possible that the main title of the report POEMS Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
- Crow-Fukase Syndrome
- PEP Syndrome
- Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
- Shimpo syndrome
- Takatsuki syndrome
POEMS syndrome is an extremely rare multisystem disorder. POEMS is an acronym that stands for (P)olyneuropathy, disease affecting many nerves; (O)rganomegaly, abnormal enlargement of an organ; (E)ndocrinopathy, disease affecting certain hormone-producing glands that help to regulate the rate of growth, sexual development, and certain metabolic functions (endocrine system); (M)onoclonal gammopathy or M proteins; and (S)kin defects. Common symptoms include progressive weakness of the nerves in the arms and legs, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), abnormally darkening of the skin (hyperpigmentation) and excessive hair growth (hypertrichosis). Endocrine abnormalities such as failure of the ovaries and testes (gonads) to function properly (primary gonadal failure) and diabetes mellitus type I may be present. Specific endocrine abnormalities associated with POEMS syndrome vary from case to case. Other important features of the disease include swelling around the optic nerve (papilledema), abnormal fluid retention, which may occur in the ankles (edema), the abdominal cavity (ascites), or around the lungs (pleural effusions), painless scars on bone x-ray (osteosclerosis), and an elevated platelet count (a blood cell responsible for clotting). Some patients may also have abnormalities in the lung function tests, which may not be noticed because of their inability to walk well due to their neuropathy.
POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulin M or IgM) in the blood. M-proteins are supposed to fight foreign substances in the body such as viruses and bacteria, but researchers suspect that they play a role in the development of POEMS syndrome. However, the specific role M-proteins play and the exact cause of POEMS syndrome is unknown. Research would suggest that a chemical called VEGF (vascular endothelial growth factor) plays an important role in this disease.
American Autoimmune Related Diseases Association, Inc.
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NIH/Developmental Endocrinology Branch
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NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
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National Institute of Neurological Disorders and Stroke (NINDS)
P.O. Box 5801
Bethesda, MD 20824
60 East 42nd Street
New York, NY 10165
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Jack Miller Center for Peripheral Neuropathy
University of Chicago
5841 South Maryland Ave, MC 2030
Chicago, IL 60637
Tel: (919) 552-9057
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