POEMS Syndrome

What is POEMS Syndrome?

POEMS syndrome is a rare disorder that affects multiple systems throughout the body. The acronym POEMS stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder, and skin changes. Polyneuropathy refers to the fact that multiple nerves are affected by the syndrome. Organomegaly essentially means that some of the person’s organs are enlarged. In the case of POEMS syndrome, this can mean the liver, spleen, and/or lymph nodes may be larger than normal.

The term endocrinopathy refers to the endrocrine system and the numerous abnormalities that can occur. These endocrine abnormalities can include diabetes mellitus, sexual dysfunction, hypothyroidism, and metabolic issues, among many others. Monoclonal plasma-proliferative disorder refers to abnormalities in the bone marrow, specifically in the blood plasma. And, of course, skin changes have to do with the skin cells and can affect pigmentation issues, nail issues, and thicker skin than normal.

There is no known cause for POEMS Syndrome. There may be a link between the levels of certain chemicals in the body like VEGF (vascular endothelial growth factor) and cytokines. Men tend to suffer from this rare medical syndrome more often than women. The condition develops in adulthood, generally in a person’s 40s or 50s but can occur as early as in their 20s. Many times the condition goes unnoticed or undiagnosed for a prolonged period of time because it is so complex and rare.

What are the Symptoms of POEMS Syndrome?

A wide array of symptoms can occur with POEMS Syndrome. The first signs that usually occur are in the nerves, specifically the peripheral nerves. A person may experience numbness and tingling, as well as weakness in the limbs. The nerve issues can spread and may even cause problems with a person’s breathing.

Other symptoms of POEMS syndrome include enlarged and swollen organs (visible on scans or in some cases, such as in the lymph nodes through the skin), impotence and sexual dysfunction, enlarged breasts, problems with menstruation, plasma tumors, other blood and cardiovascular issues, and the aforementioned skin pigmentation and thickness problems.

POEMS Syndrome Causes

POEMS Syndrome is an extremely infrequent multisystemic blood disorder that escalates the production of plasma cells in people. Symptoms associated with this syndrome range from fever, diarrhea and renal deficiency to papilledema (optic disc swelling caused by increased pressure in or around the brain), thrombosis, ascites (accumulation of fluid in the peritoneal cavity), etc. POEMS is an acronym that stands for the following symptoms: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma proliferative disorder and Skin changes. Patients diagnosed with POEMS Syndrome typically suffer from at least 3 of these five symptoms. While the cause for POEMS Syndrome is not well known, the earliest case of it was recorded in 1956. Organs that are commonly affected are the liver, spleen and lymph nodes. The majority of patients have been known to have more than one endocrine anomaly, as well as glucose intolerance, hypothyroidism and hypoparathyroidism. Some studies have shown unusually increased levels of vascular endothelial growth factor (VEGF) in patients’ serum suffering from POEMS Syndrome. More research needs to be concluded in order to fully understand the causes of this disease.

How is POEMS Syndrome Treated?

Oftentimes, treatment depends on the exact symptoms that occur in the individual. A team of specialists often work together to deal with the multiple systems affected by the condition, but the primary treatment often focuses on blood plasma issues and the associated tumors or lesions. This can involve both radiation therapy and chemotherapy. Supportive treatments like occupational and physical therapy as well as respiratory therapy can help with other symptoms as needed.

POEMS Syndrome Prevention

POEMS Syndrome can spread rapidly if left untreated. Treatment of this syndrome calls upon the combined efforts of multiple specialists. There are two ways in which POEMS Syndrome is commonly tackled. First, treatment is directed towards the plasma cell disorder. Second, specific symptoms that occur in individual patients are treated. Radiotherapy and excision of localized lesions found throughout the patient’s body may for a time (and sometimes for good) cause the symptoms of POEMS Syndrome to go into remission. Chemotherapy may ease symptoms in many cases, especially when a patient is also treated with a peripheral blood stem cell transplant. Experimental treatments of POEMS Syndrome have been discovered in the early 2000s. These new drugs are used with extreme discretion due to the fact that they may cause peripheral neuropathy (the main symptom in POEMS Syndrome).

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Last Reviewed:
October 08, 2016
Last Updated:
January 09, 2018