POEMS syndrome is a rare paraneoplastic syndrome caused by an abnormal clone of plasma cells. Its name is an acronym for its major features: polyneuropathy, organomegaly, endocrinopathy, myeloma protein, an abnormal immunoglobulin, and skin changes; a related name, PEP syndrome, covers polyneuropathy, endocrinopathy and plasma cell dyscrasia. Its symptoms arise from humoral factors, including hormones, cytokines and immunoglobulins, released by the neoplastic plasma cells and by the body's immune response to them, with many features traced to the abnormal myeloma proteins and specific cytokines the malignant cells produce. It is also known as Crow-Fukase syndrome, osteosclerotic myeloma and Takatsuki syndrome. It falls under oncology, usually begins in the fifth or sixth decade of life with an average age of onset around 50, is chronic and rare, affects up to twice as many men as women, and carries a median survival of about 13.8 years.
Facts
Classification
ICD-10 Chapter Communicable or Non-communicable Transmission Route First Described Sources
1. National Library of Medicine: MeSH Descriptor Data: POEMS Syndrome
National Library of MedicineMeSH tree number C10.668.829.800.700
A multisystemic disorder characterized by a sensorimotor polyneuropathy
MeSH tree number C20.683.780.750
A multisystemic disorder characterized by a sensorimotor polyneuropathy
View the Source 2. Wikidata: POEMS syndrome
3. POEMS syndrome (Wikipedia)
History section
Crow, working in Bristol, first described the combination of osteosclerotic myeloma, polyneuropathy and various unusual features (such as pigmentation and clubbing) in two patients aged 54 and 67, in 1956.
Lead
POEMS syndrome (also termed osteosclerotic myeloma, Crow-Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells.
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