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.
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.
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.