POEMS syndrome

What causes POEMS syndrome?

The cause of POEMS syndrome is not well understood. POEMS syndrome is a paraneoplastic disorder, which means that the signs and symptoms of the syndrome appear as a manifestation of the plasma cell disorder that the patient has (most patients are seen with osteosclerotic myeloma or monoclonal gammopathy of unknown significance); however, the mechanism by which this occurs is unknown. It is associated with a chronic overproduction of some substances known as pro-inflammatory cytokines (small molecules that act as messengers between cells to promote inflammation). These include interleukins (IL-1b, IL6) which are cytokines that help regulate immune responses, TNFα (Tumor Necrosis Factor alfa is a cytokine which is involved in the inflammatory process) and vascular endothelial growth factor (VEGF – a powerful vasodilator that may cause leaky blood vessels) which seem to be important in this disorder.

Last updated on 05-01-20

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 gynecomastia 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:

  • Organomegaly (enlargement of the spleen, liver, or lymph nodes)
  • Extravascular volume overload (peripheral edema, ascites, or pleural effusion)
  • Endocrinopathy (excluding diabetes mellitus or hypothyroidism)
  • Skin changes
  • Papilledema
  • Thrombocytosis or polycythemia

Many affected people are initially misdiagnosed as having other disorders, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). In addition, a number of conditions are associated with a plasma cell disorder and polyneuropathy (with or without osteosclerotic bone lesions), and need to be distinguished from POEMS syndrome.

Last updated on 05-01-20

What is the long-term outlook for people with 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.

Last updated on 05-01-20

Where To Start

POEMS syndrome

The MayoClinic Web site provides information on this topic. Click on MayoClinic to access the information page on POEMS syndrome.

Last updated on 04-27-20

Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: Neuropathy Association 60 East 42nd Street Suite 942
New York, NY, 10165, United States
Phone: 212-692-0662 Email: info@neuropathy.org Url: http://www.neuropathy.org/
Chan JL. POEMS Syndrome Medscape Reference. March 6, 2013; Reference Link

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