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
References
Mayo Clinic
Genetic and Rare Diseases Information Center (GARD)
Neurology
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