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: email@example.com Internet: http://www.aarda.org/
NIH/Child Health and Human Development - Section on Endocrinology and Genetics Bldg 31, Room 2A32, MSC 2425 31 Center Drive Bethesda, MD 20892-2425 Tel: (301)402-1998 Fax: (301)402-2984 Email: firstname.lastname@example.org Internet: http://segen.nichd.nih.gov/index.html
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: email@example.com Internet: http://www.neuropathy.org
Genetic and Rare Diseases (GARD) Information Center PO Box 8126 Gaithersburg, MD 20898-8126 Tel: (301)251-4925 Fax: (301)251-4911 Tel: (888)205-2311 TDD: (888)205-3223 Internet: http://rarediseases.info.nih.gov/GARD/
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: firstname.lastname@example.org Internet: http://www.autoimmunitycommunity.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email email@example.com
Last Updated: 9/7/2012 Copyright 1991, 2000, 2002, 2003, 2004, 2008, 2012 National Organization for Rare Disorders, Inc.
How this information was developed to help you make better health decisions.
Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.