Important 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.
Summary 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 sexual function, and certain metabolic functions; (M)onoclonal gammopathy or M proteins; and (S)kin abnormalities. Common symptoms include progressive weakness of the nerves in the legs and arms, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), enlarged lymph nodes, abnormally darkening of the skin (hyperpigmentation), thickening of the skin 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 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.
Introduction 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 immunoglobulins) in the blood. Immunoglobulins in health fight infection. 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. 22100 Gratiot Ave. Eastpointe, MI 48021 Tel: (586)776-3900 Fax: (586)776-3903 Tel: (800)598-4668 Email: firstname.lastname@example.org Internet: http://www.aarda.org/
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NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse One AMS Circle Bethesda, MD 20892-3675 USA Tel: (301)495-4484 Fax: (301)718-6366 Tel: (877)226-4267 TDD: (301)565-2966 Email: NIAMSinfo@mail.nih.gov Internet: http://www.niams.nih.gov/
NIH/National Institute of Neurological Disorders and Stroke P.O. Box 5801 Bethesda, MD 20824 Tel: (301)496-5751 Fax: (301)402-2186 Tel: (800)352-9424 TDD: (301)468-5981 Internet: http://www.ninds.nih.gov/
Neuropathy Association 60 East 42nd Street Suite 942 New York, NY 10165 USA Tel: (212)692-0662 Fax: (212)692-0668 Email: firstname.lastname@example.org Internet: http://www.neuropathy.org
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Center for Peripheral Neuropathy University of Chicago 5841 South Maryland Ave, MC 2030 Chicago, IL 60637 Tel: (773)702-5659 Fax: (773)702-5577 Internet: http://peripheralneuropathycenter.uchicago.edu/
AutoImmunity Community Email: email@example.com Internet: http://www.autoimmunitycommunity.org
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