What is POEMS Syndrome?

POEMS syndrome refers to a rare disorder that causes a buildup of proteins in the organs and tissue cells throughout various parts of the body. This disorder also causes varying degrees of peripheral nerve damage. While POEMS syndrome can affect anyone at any age, it most commonly affects men over the age of 50 years old.

This disorder is labeled POEMS syndrome as an acronym representing each of the symptoms relative to this condition. The five major symptoms associated with this disorder are peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Although each of these represents a common symptom of this rare condition, not all symptoms need be present for a person to be diagnosed with this syndrome. A thorough examination of a patient presenting some or all of these symptoms is needed before a final diagnosis is made.

The peripheral neuropathy common in this syndrome may affect several nerves at the same time. Organomegaly refers to enlarged organs, which is usually either the spleen or the liver and may affect both. Abnormal enlargement may also occur in the lymph nodes of people afflicted with this syndrome.

The endocrinopathy common in POEMS syndrome produces a change or a disruption in a person’s hormone levels, which commonly leads to hormonal imbalance. Endocrinopathy may also affect sexual organs, such as preventing the ovaries or the testicles from functioning properly. When monoclonal gammopathy occurs, it causes the production of immunoglobulin from bone marrow cells, which have grown out of control. Skin changes, which commonly occur in POEMS syndrome, include skin thickening and changes in pigmentation.

More specific symptoms of POEMS syndrome include fatigue, progressive weakness in the extremities, edema, neuropathy in the hands and feet and vision changes. Many with this syndrome are also affected by Type 1 diabetes. Although some people do not experience all of these symptoms, most people affected by this syndrome do experience peripheral neuropathy symptoms, as well as monoclonal gammopathy.

Although it is a very rare disorder, cases of POEMS syndrome are found in various parts of the world. It is found most frequently, however, in aging men in Japan where it is also known as Crow-Fukase syndrome. Other names for POEMS syndrome used interchangeably are Takatsuki disease, PEP syndrome and Shimpo syndrome. There is research to suggest that this syndrome may actually be a very rare blood disorder of unknown origin. Other research suggests that it falls under the classification of immune system disorders.