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POEMS syndrome

Evidence: Unconfirmed

Names

POEMS syndrome
Crow-Fukase syndrome
osteosclerotic myeloma
PEP syndrome
Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions
Shimpo syndrome
Takatsuki syndrome

Description

POEMS is an acronym that stands for the disorder’s features, which may include Polyneuropathy (disease affecting many nerves), Organomegaly (abnormal enlargement of an organ), Endocrinopathy (diseases affecting certain hormone-producing glands that help to regulate sexual function), Monoclonal gammopathy and Skin Changes. POEMS syndrome is a rare blood disorder where there is an uncontrolled growth of a single clone of plasma cells, which results in abnormal accumulation of immunoglobulins in the blood.

Patient Groups

None

Bloggers

None

Prevalence

US Cases: 950 [Wang 2019].

Typical Age of onset

21-84 years. Median age is 54 years.

Symptoms

  • enlarged liver or spleen

  • skin abnormalities - darkening of the skin, excessive hair growth, hardening and thickening of the skin

  • excessive sweating

  • progressive weakness in the legs and arms

  • enlarged lymph nodes

  • swelling around the optic nerve

  • abnormal fluid retention that leads to swelling of the ankles and abdomen