POEMS syndrome
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Random blog day boo yah. As you should know Fridays is random blog day were we talk anything and everything and pretty much run with it. Today's blog is on Poems Syndrome.
POEMS Syndrome also termed and known as PEP Syndrome, Crow—Fukase Syndrome, Osteosclerotic Myeloma or Takatsuki disease is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells. POEMS is an acronym for some of the diseases major signs and symptoms (Polyneuropathy, Organomegaly, Endocrinopathy, Myeloma protein and Skin changes) as is PEP (Polyneuropathy, endocrinopathy, plasma cell dyscrasia).
The signs and symptoms of most neoplasms are due to the mass effects of the invasion and destruction of neoplasms cells. The signs and symptoms of a cancer causing paraneoplastic syndrome often result from the release of humoral factors, aka hormones, cytokines or immunoglobulins, usually by the syndrome's neoplastic cells and/or the response of the immune system to the neoplasm. POEMS syndromes signs and symptoms are due in part to the release of an aberrant immunoglobulin as in a myeloma protein, certain cytokines by the malignant plasma cells are causes too.
The signs and symptoms for POEMS Syndrome are varied which does lead to long delays between initial symptoms and a diagnosis usually 13-18 months on average. Whilst some of the symptoms are indicated by the POEMS acronym, the PEST acronym covers other symptoms of the disease. PEST stands for Paphilledema, evidence of Extravascular volume overload (ascites, pleural effusion etc.), Sclerotic bone lesions and Thrombocytosis/erythocytosis (Ie increased blood platelets and red blood cells). Abnormal lung function (restrictive lung disease, pulmonary hypertension and impaired lung diffusion capacity), a Tendency towards Leukocytosis, blood clot formation and high blood levels of the Cytokine VEGF (Vascular Endothelial Growth Factor) are also other symptoms of the disease that also cross over with signs and symptoms of Multicentric Castleman disease.
POEMS Syndrome typically begins in middle age –the average age of onset is 50-- and effects twice as many men as it does women.
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THE WEBSITE BIBLIOGRAPHY:
https://en.wikipedia.org/wiki/POEMS_syndrome
https://www.myeloma.org.uk/understanding-myeloma/related-conditions/poems/
https://rarediseases.org/rare-diseases/poems-syndrome/
https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678
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