POEMS syndrome (patient information)

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For the WikiDoc page for this topic, click POEMS syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]


POEMS syndrome is a rare, mulitisystem disorder. POEMS stands for the disorder's features, which may include polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Signs and symptoms may include progressive sensorimotor polyneuropathy (weakness, numbness and tingling); enlarged liver, spleen, and/or lymph nodes; a disorder of the endocrine glands (often with multiple abnormalities); an increase in the number of plasma cells in blood (mature B type cells); and darkening of the skin (hyperpigmentation). Various other symptoms may also occur and may vary among affected people. The underlying cause of the disorder is not well understood. POEMS syndrome is a chronic disorder, with a median survival time of 8-14 years. There is no standard treatment; management depends on the underlying plasma cell disorder and may include radiation therapy, chemotherapy, and/or hematopoietic cell transplantation.

What are the causes of POEMS syndrome?

How is POEMS syndrome diagnosed?

  • People who are suspected of having POEMS syndrome should first have their medical history evaluated and undergo a complete physical exam. Features suggestive of POEMS syndrome in the history include complaints of neurological symptoms, skin changes, signs of extravascular volume overload, and symptoms suggestive of an endocrine disorder (such as vreast development in males or irregular periods). A detailed physical exam should include an eye exam for papilledema; a neurologic exam; evaluation for enlargement of organs (organomegaly); examination of the skin; and evaluation for the presence of peripheral edema (swelling due to fluid accumulation), pleural or pericardial effusion (fluid build-up around the lungs or heart), ascites, clubbing (enlargement of the fingertips), heart failure, and cardiomyopathy.
  • A diagnosis of POEMS syndrome requires both the presence of polyneuropathy and a monoclonal plasma cell proliferative disorder, plus the presence of at least one major and one minor criterion on a physical exam, imaging, or laboratory evaluation.

Major criteria include:

Minor criteria include:

What is the prognosis of POEMS syndrome?

  • POEMS syndrome is a chronic condition. In many cases, there is progressive peripheral neuropathy that eventually causes mobility disability. The overall median survival has been estimated to be 13.7 years, while those with clubbing or extravascular volume overload may have median survivals of 2.6 and 6.6 years, respectively. Those who receive radiation therapy with a good response to treatment are thought to have longer survival. Death usually occurs from lack of nourishment or bronchopneumonia.

Conditions with similar signs and symptoms

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to POEMS syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Organizations Providing General Support

  1. American Autoimmune Related Diseases Association (AARDA) Website: https://www.aarda.org/
  2. Neuropathy Association Website: http://www.neuropathy.org/