An Overview of POEMS Syndrome

Laboratory technician holding test tubes containing blood samples
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POEMS syndrome is a rare blood disorder that affects many parts of the body. It affects both men and women and usually begins after age 50, although it may occur at any age. It is not known exactly how often POEMS syndrome occurs because it is difficult to diagnose correctly.

Symptoms

The acronym POEMS stands for:

  • P: Polyneuropathy, meaning nerve symptoms such as numbness, tingling, and/or weakness of the hands and feet
  • O: Organomegaly, meaning enlarged organs, usually the liver, spleen, or lymph nodes. About 15% of individuals with POEMS syndrome have Castleman disease (a lymph node disorder).
  • E: Endocrinopathy, meaning changes in hormone production. Symptoms such as diabetes, impotence, stopped menstrual periods (amenorrhea), low thyroid levels (hypothyroidism), and/or breast development in males (gynecomastia) may be present.
  • M: Monoclonal gammopathy or monoclonal plasma proliferative disorder, both of which mean abnormal blood protein cells
  • S: Skin changes such as increased skin pigment (hyperpigmentation); increased body hair (hypertrichosis) on the face, limbs, and chest; skin thickening and tightening, swelling (edema) of the legs and feet, whitening of the nails.

People with POEMS syndrome may also experience, fatigue, generalized aches and pains, and changes in vision, among other symptoms.

Causes

The cause of POEMS syndrome isn't known, though people with the disease have an increased number of plasma cells. These cells produce an abnormal amount of a type of protein, which can damage other parts of the body.

Diagnosis

A diagnosis of POEMS syndrome may be made based on a patient's physical examination and his/her medical history. Blood tests such as a complete blood cell count (CBC) and serum protein immunoelectrophoresis are done to look for abnormalities, as is urine testing. Thyroid hormone levels, blood glucose levels, and estrogen levels in the blood are tested to identify abnormalities. A bone marrow biopsy and/or bone x-rays may be performed to aid in diagnosis.

In addition, diagnostic tests may 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 person is considered to have POEMS syndrome if the “P” (polyneuropathy) and “M” (abnormal blood proteins) are present along with either sclerotic (scarring) bone lesions or Castleman disease. Many individuals will have other symptoms of the syndrome as well. Because many physicians are not familiar with POEMS syndrome, the abnormal blood test results often result in a misdiagnosis of multiple myeloma, or the polyneuropathy symptoms lead to a misdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP).

Treatment

The treatment of POEMS syndrome depends on the treatment of the underlying blood cell disorder. Most individuals receive a combination of treatments such as:

  • Radiation therapy
  • Corticosteroid medications such as prednisone
  • Chemotherapy
  • Hormone replacement of hormones which are too low
  • Physical therapy
  • Bone marrow transplant

Specialists such as a neurologist (nervous system doctor), hematologist (blood disorders doctor), dermatologist (skin doctor), an endocrinologist (hormone gland doctor) are usually involved in the care of those with POEMS syndrome.

A Word From Verywell

POEMS syndrome is a chronic condition. In many cases, there is progressive peripheral neuropathy that eventually causes disability. The overall median survival has been estimated to be 13.7 years. Those who receive and respond well to radiation therapy are thought to fare better. If you experience symptoms including polyneuropathy and skin changes, make an appointment with your doctor for an evaluation.

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Article Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial policy to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  • Dispenzieri, Angela, Robert A. Kyle, Martha Q. Lacy, S. Vincent Rajkumar, Terry M. Therneau, Dirk R. Larson, Philip R. Greipp, Thomas E. Witzig, Rita Basu, Guillermo A. Suarez, Rafael Fonseca, John A. Lust, & Morie A. Gertz. POEMS syndrome: definitions and long-term outcome. Blood 101(2003): 2496-2506.

  • Dispenzieri, Angela. POEMS Syndrome. Hematology 2005(2005): 360-367.

  • Genetic and Rare Diseases Information Center. POEMS Syndrome.