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POEMS Syndrome

Blood Disorder Causes Many Symptoms

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Updated May 25, 2007

The nervous system is one part of the body affected by POEMS syndrome

The nervous system is one part of the body affected by POEMS syndrome

A.D.A.M.
POEMS syndrome is a blood disorder that affects many parts of the body and causes many symptoms. 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. The cause is unknown.

Symptoms

The acronym POEMS stands for:
  • Ppolyneuropathy, 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 percent 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.
Individuals may also experience, fatigue, generalized aches and pains, or changes in vision, among other symptoms.

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.

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 for 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), and endocrinologist (hormone gland doctor) are usually involved in the care of an individual with POEMS syndrome.

Sources:

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

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.

"Symptoms of POEMS Syndrome." Treatment of POEMS Syndrome at Mayo Clinic. The Mayo Clinic. 22 May 2007 <http://www.mayoclinic.org/poems/symptoms.html>.

Chan, Joanna, & Wingfield Rehmus. "POEMS Syndrome." eMedicine. 15 Dec 2006. WebMD. 22 May 2007 <http://www.emedicine.com/derm/topic771.htm>.

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