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POEMS Syndrome

Abstract

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NORD is very grateful to Angela Dispenzieri, MD, Consultant, Division of Hematology, Professor of Medicine, Laboratory Medicine, and Pathology, Mayo Clinic, for assistance in the preparation of this report.

Synonyms of POEMS Syndrome

  • Crow-Fukase syndrome
  • osteosclerotic myeloma
  • PEP syndrome
  • polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
  • Shimpo syndrome
  • Takatsuki syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
POEMS syndrome is an extremely rare multisystem disorder. POEMS is an acronym that stands for (P)olyneuropathy, disease affecting many nerves; (O)rganomegaly, abnormal enlargement of an organ; (E)ndocrinopathy, disease affecting certain hormone-producing glands that help to regulate sexual function, and certain metabolic functions; (M)onoclonal gammopathy or M proteins; and (S)kin abnormalities. Common symptoms include progressive weakness of the nerves in the legs and arms, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), enlarged lymph nodes, abnormally darkening of the skin (hyperpigmentation), thickening of the skin and excessive hair growth (hypertrichosis). Endocrine abnormalities such as failure of the ovaries and testes (gonads) to function properly (primary gonadal failure) and diabetes mellitus may be present. Specific endocrine abnormalities associated with POEMS syndrome vary from case to case. Other important features of the disease include swelling around the optic nerve (papilledema), abnormal fluid retention, which may occur in the ankles (edema), the abdominal cavity (ascites), or around the lungs (pleural effusions), painless scars on bone x-ray (osteosclerosis), and an elevated platelet count (a blood cell responsible for clotting). Some patients may also have abnormalities in the lung function tests, which may not be noticed because of their inability to walk well due to their neuropathy.

Introduction
POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulins) in the blood. Immunoglobulins in health fight infection. However, the specific role M-proteins play and the exact cause of POEMS syndrome is unknown. Research would suggest that a chemical called VEGF (vascular endothelial growth factor) plays an important role in this disease.

Organizations related to POEMS Syndrome

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder (e.g., polyneuropathy, skin abnormalities, etc.)

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