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Difference between revisions of "POEMS syndrome"
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diagnostic criteria
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'''POEMS syndrome''' is a constellation of findings:<ref name=pmid18477219>{{cite journal |author=Yuri T, Yamazaki F, Takasu K, Shikata N, Tsubura A |title=Glomeruloid hemangioma |journal=Pathol. Int. |volume=58 |issue=6 |pages=390–5 |year=2008 |month=June |pmid=18477219 |doi=10.1111/j.1440-1827.2008.02241.x |url=}}</ref> | '''POEMS syndrome''' is a constellation of findings, best thought of as a paraneoplastic syndrome due to clonal plasma cells:<ref name=pmid18477219>{{cite journal |author=Yuri T, Yamazaki F, Takasu K, Shikata N, Tsubura A |title=Glomeruloid hemangioma |journal=Pathol. Int. |volume=58 |issue=6 |pages=390–5 |year=2008 |month=June |pmid=18477219 |doi=10.1111/j.1440-1827.2008.02241.x |url=}}</ref> | ||
*Polyneuropathy. | *Polyneuropathy. | ||
*Organomegaly. | *Organomegaly. | ||
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*M-protein. | *M-protein. | ||
*Skin changes. | *Skin changes. | ||
Diagnostic criteria requires both mandatory criteria and at least one each from major and minor criteria:<ref name=pmid31012139>{{cite journal |vauthors=Dispenzieri A |title=POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=94 |issue=7 |pages=812–827 |date=July 2019 |pmid=31012139 |doi=10.1002/ajh.25495 |url=}}</ref> | |||
Mandatory: | |||
1. Polyneuropathy (typically demyelinating) | |||
2. Monoclonal plasma cell proliferation (usually lambda) | |||
Major: | |||
3. [[Castleman disease]] | |||
4. Sclerotic bone lesions | |||
5. Vascular endothelial growth factor elevation | |||
Minor: | |||
6. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy) | |||
7. Extravascular volume overload | |||
8. Endocrinopathy | |||
9. Sking changes | |||
10. Papilloedema | |||
11. Thrombocytosis/polycythemia | |||
POEMS syndrome is unusual in causing osteosclerotic bone deposits, so-called osteosclerotic myeloma, in contrast to the lytic lesions seen in more conventional [[plasma cell myeloma]]. | POEMS syndrome is unusual in causing osteosclerotic bone deposits, so-called osteosclerotic myeloma, in contrast to the lytic lesions seen in more conventional [[plasma cell myeloma]]. | ||