(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)
(See also Polyglandular Deficiency Syndrome.)
POEMS syndrome is a rare disorder in which more than one endocrine gland is dysfunctional.
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. In certain plasma cell disorders, abnormal plasma cells multiply and produce excessive amounts of an antibody that damages organs.
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 (the abnormal blood antibodies), and skin changes.
People may have the following:
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
Skin abnormalities, such as darkening and thickening of the skin, hairiness, and small bright red patches (angiomas)
Fluid may accumulate in the legs, abdomen, 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 fever.
POEMS syndrome is diagnosed based on the symptoms and the findings during a doctor's physical examination.
Treatment consists of radiation therapy and chemotherapy to reduce the number of abnormal plasma cells followed by autologous hematopoietic stem cell transplantation. About 60% of affected people survive for 5 years after diagnosis.