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POEMS Syndrome

(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)

By

Jennifer M. Barker

, MD, Children's Hospital Colorado, Division of Pediatric Endocrinology

Last full review/revision Mar 2021| Content last modified Mar 2021
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POEMS syndrome is a rare disorder in which more than one endocrine gland is dysfunctional.

Endocrine glands Endocrine Glands The endocrine system consists of a group of glands and organs that regulate and control various body functions by producing and secreting hormones. Hormones are chemical substances that affect... read more 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 Overview of Plasma Cell Disorders Plasma cell disorders are uncommon. They begin when a single plasma cell multiplies excessively. The resulting group of genetically identical cells (called a clone) produces a large quantity... read more , 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 (abnormal blood antibodies)

  • Skin changes

People may have the following:

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 presence of certain symptoms and findings during a doctor's physical examination, and certain laboratory test abnormalities in antibody and hormone levels.

Treatment of POEMS Syndrome

  • Radiation therapy

  • Chemotherapy

Treatment consists of radiation therapy and chemotherapy to reduce the number of abnormal plasma cells sometimes followed by autologous stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more . About 60% of affected people survive for 5 years after diagnosis.

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