POEMS Syndrome
The average survival time for POEMS syndrome is 8 to 14 years.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is blood disorder.

In this rare and chronic condition, unusual plasma cells prompt elevated degrees of antibody proteins to clump in the blood. A large number of these proteins in the blood damage nerves and affect other organ systems, such as the skin, liver, spleen, and gonads.

The average survival time for those with POEMS syndrome is 8 to 14 years.

What causes POEMS symptoms?

The cause for POEMS syndrome is still unknown.

Individuals with the disorder have an excess of plasma cells and increased blood levels of vascular endothelial growth factor, a protein that stimulates the formation of blood vessels. 

Levels of chemicals in the blood, such as cytokines interleukin-6, interleukin-1, and TNF-alpha, are also high.

What are the signs and symptoms of POEMS syndrome?

POEMS syndrome gets its name from the most common signs and symptoms experienced by individuals with the disorder.

  • Polyneuropathy (P). Numbness, shivers, and weakness in the legs, and, over the long run, may affect the hands. This is a fundamental element in the diagnosis of POEMS disorder.
  • Organomegaly (O). Enlarged spleen, liver, or lymph nodes.
  • Endocrinopathy or edema (E). Abnormal hormone levels can result in hypothyroidism, diabetes, sexual issues, weakness, swelling in the limbs, and issues with digestion and other bodily functions.
  • Monoclonal gammopathy (M). Abnormal bone marrow cells (plasma cells) produce a protein (monoclonal protein) that can be found in the blood. This is another important feature of POEMS disorder. Monoclonal protein is frequently connected with abnormal bone hardening or thickening.
  • Skin changes (S). Skin darkening, red spots, perhaps thicker skin, and increased facial or leg hair.

The symptoms vary depending on the part of the body that has been affected, but may include:

  • Fluid retention all through the body
  • Numbness
  • Pain
  • Reproductive system problems
  • Weakness
  • Vision issues
  • Feeling tired
  • Loss of muscle

How is POEMS syndrome diagnosed?

POEMS syndrome diagnosis includes inquiry about the clinical history and a few other tests, such as:

  • Biopsy: The doctor takes a sample of bone marrow and looks for any abnormal plasma cells.
  • Electromyogram: A unique test to measure muscle functions in response to nerve stimulation.
  • Blood or urine tests: A sample of blood or urine is collected to check for protein levels and other substances.
  • Imaging: X-ray and computed tomography scans allow the doctor to search for bone lesions (abnormal thickening or hardening).
  • Other tests: These might include lung function tests and echocardiograms depending on the symptoms.

How is POEMS syndrome treated?

Treatment for POEMS syndrome involves managing the symptoms and minimizing complications because there is no standard treatment for this disease.

The treatment for POEMS may include:

  • Chemotherapy: Anticancer medications slow or kill the abnormal plasma cells. This treatment is similar to cancer treatments. This is effective and, by and large, well-tolerated and does not have many side effects.
  • Immunotherapy: Immunotherapy helps the immune system to kill off the abnormal plasma cells.
  • Radiation therapy: Doctors utilize high degrees of radiation to kill the unwanted plasma cells.
  • Physical therapy: Helps manage mobility issues brought about by neuropathy.
  • Hematopoietic cell transplantation: Replenishes healthy blood cells in the patient’s body.
  • Other medication: Steroids and additional diuretics (to ease swelling) may likewise be prescribed.

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Medically Reviewed on 11/29/2021
References
Image Source: iStock Images

Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003 Apr 1;101(7):2496-506. https://ashpublications.org/blood/article/101/7/2496/106657/POEMS-syndrome-definitions-and-long-term

Chan JL. POEMS Syndrome. Medscape. https://emedicine.medscape.com/article/1097031-overview