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POEMS Syndrome

(Crow-Fukase Syndrome)

By Jennifer M. Barker, MD, Children's Hospital Colorado, Division of Pediatric Endocrinology

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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 (see Endocrine Glands). 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 (see Overview of 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:

  • 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)

  • 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, pleural space (causing difficulty breathing and chest pain), or near the optic nerve (causing 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 chemotherapy and radiation therapy to reduce the number of abnormal plasma cells followed by autologous hematopoietic stem cell transplantation (see Stem Cell Transplantation). About 60% of affected people survive for 5 years after diagnosis.

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