POEMS syndrome is a rare disorder in which more than one endocrine gland is dysfunctional.
(See also Polyglandular Deficiency Syndromes.)
Endocrine glands are organs that secrete one or more specific hormones. POEMS syndrome is probably caused by antibodies (immunoglobulins) that circulate in the blood and damage organs, particularly endocrine glands. Antibodies are produced by cells called plasma cells. POEMS can develop in people with certain plasma cell disorders, in which abnormal plasma cells multiply and produce excessive amounts of an antibody that damages organs. POEMS sometimes develops in people with cancer. It is not caused by the cancer itself but is a condition that occurs only when a person already has cancer (called a paraneoplastic syndrome).
Symptoms of POEMS Syndrome
POEMS syndrome is named for the signs and symptoms that commonly occur in affected people:
Polyneuropathy (nerve damage)
Organomegaly (enlargement of the liver, spleen, and lymph nodes)
Endocrinopathy (abnormal hormone levels)
Monoclonal gammopathy (abnormal blood antibodies)
Skin changes
People may have the following:
Liver enlargement
Swollen lymph glands
Low levels of testosterone (hypogonadism)
Diabetes mellitus type 2
Low levels of thyroid hormone (hypothyroidism)
High levels of parathyroid hormone (hyperparathyroidism)
Underactive adrenal glands (Addison disease)
Excess production of some antibodies (gammopathy)
Skin abnormalities, such as darkening and thickening of the skin, hairiness, and small bright red patches (angiomas)
Areas of bone destruction
Fluid may accumulate in the legs, abdomen, or space between the layers of the membrane surrounding the lungs (called the pleural space), or near the optic nerve. Fluid accumulation in the pleural space causes difficulty breathing and chest pain. Fluid that accumulates near the optic nerve and presses on it causes vision problems.
People may also have chronic and intermittent fever.
Diagnosis of POEMS Syndrome
POEMS syndrome is diagnosed based on the presence of certain symptoms and findings during a doctor's physical examination and on abnormalities in certain antibody and hormone levels detected on blood tests.
Treatment of POEMS Syndrome
Radiation therapy
Chemotherapy
Treatment consists of chemotherapy and/or radiation therapy to reduce the number of abnormal plasma cells. Sometimes autologous stem cell transplantation is done after radiation therapy and chemotherapy. About 60% of affected people survive for 5 years after diagnosis.
