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Genetic and Rare Diseases Information Center (GARD)

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POEMS syndrome


Other Names for this Disease
  • Crow-Fukase syndrome
  • Polyneuropathy organomegaly
  • Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes syndrome
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Overview


POEMS syndrome is a rare blood disorder. It is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma proliferative disorder, and Skin changes.[1] 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.[2] Many people with POEMS syndrome have diabetes (20-50%) and primary gonadal failure (55-70%).[2] 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.[1] 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.[2] Currently the exact cause of POEMS syndrome is unknown.[2] Researchers believe the syndrome involves an overgrowth of bone marrow cells (plasma cells), which produce chemicals that damage other parts of the body.[1]
Last updated: 2/18/2013

References

  1. POEMS syndrome. MayoClinic.org. http://www.mayoclinic.org/poems-syndrome/. Accessed 2/18/2013.
  2. Barker JM, Gottlieb PA, Eisenbarth GS. The immunoendocrinopathy syndromes. In: Kronenberg HM, Melmed S, Polonsky KS, Larsen PR eds. Williams Textbook of Endocrinology, 11th ed. Philadelphia, PA: Saunders; 2008;
Your Questions Answered
by the Genetic and Rare Diseases Information Center

1 question(s) from the public on POEMS syndrome have been answered. See questions and answers. You can also submit a new question.

Basic Information

  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
  • TheDoctorsDoctor provides information on this condition. TheDoctorsDoctor is an online resource developed by pathologists. Click on TheDoctorsDoctor to view the information page.
  • The Merck Mauals provides information on polyglandular deficiency syndromes which includes information on POEMS syndrome. The Merck Manuals are a series of healthcare books for medical professionals and consumers.
  • The MayoClinic Web site provides information on this topic. Click on MayoClinic to access the information page on POEMS syndrome.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss POEMS syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

  • Dispenzieri A, Gertz MA. POEMS syndromeOrphanet Encyclopedia. 2005.