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POEMS Syndrome

Abstract

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NORD is very grateful to Angela Dispenzieri, MD, Consultant, Division of Hematology, Professor of Medicine, Laboratory Medicine, and Pathology, Mayo Clinic, for assistance in the preparation of this report.

Synonyms of POEMS Syndrome

Crow-Fukase syndrome
osteosclerotic myeloma
PEP syndrome
polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
Shimpo syndrome
Takatsuki syndrome

Disorder Subdivisions

No subdivisions found.

General Discussion

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.

Organizations related to POEMS Syndrome

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder (e.g., polyneuropathy, skin abnormalities, etc.)

American Autoimmune Related Diseases Association, Inc.
- 22100 Gratiot Ave.
- Eastpointe, MI 48021
- Phone #: 586-776-3900
- 800 #: 800-598-4668
- e-mail: aarda@aarda.org
- Home page: http://www.aarda.org/
AutoImmunity Community
- Phone #: N/A
- 800 #: N/A
- e-mail: moderator@autoimmunitycommunity.org
- Home page: http://www.autoimmunitycommunity.org
Center for Peripheral Neuropathy
- University of Chicago
- 5841 South Maryland Ave, MC 2030
- Chicago, IL 60637
- Phone #: 773-702-5659
- 800 #: N/A
- e-mail: N/A
- Home page: http://peripheralneuropathycenter.uchicago.edu/
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Phone #: 301-251-4925
- 800 #: 888-205-2311
- e-mail: N/A
- Home page: http://rarediseases.info.nih.gov/GARD/
Neuropathy Association
- 60 East 42nd Street
- Suite 942
- New York, NY 10165 USA
- Phone #: 212-692-0662
- 800 #: N/A
- e-mail: info@neuropathy.org
- Home page: http://www.neuropathy.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
- Phone #: 301-402-1998
- 800 #: --
- e-mail: stratakc@exchange.nih.gov
- Home page: 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
- Phone #: 301-495-4484
- 800 #: 877-226-4267
- e-mail: NIAMSinfo@mail.nih.gov
- Home page: http://www.niams.nih.gov/
NIH/National Institute of Neurological Disorders and Stroke
- P.O. Box 5801
- Bethesda, MD 20824
- Phone #: 301-496-5751
- 800 #: 800-352-9424
- e-mail: N/A
- Home page: http://www.ninds.nih.gov/

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