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Difference between revisions of "POEMS syndrome"
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typically osteosclerotic
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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. | ||
| Line 5: | Line 5: | ||
*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 |authors=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:'' | |||
# Polyneuropathy (typically demyelinating). | |||
# Monoclonal plasma cell proliferation (usually lambda). | |||
''Major:'' | |||
# [[Castleman disease]] | |||
# Sclerotic bone lesions. | |||
# Vascular endothelial growth factor elevation. | |||
''Minor:'' | |||
# Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy). | |||
# Extravascular volume overload. | |||
# Endocrinopathy. | |||
# Skin changes. | |||
# Papilloedema. | |||
# Thrombocytosis/polycythemia. | |||
==Pathology== | ==Pathology== | ||
*The [[pathologist]] may come across an undiagnosed case in the form of a ''[[glomeruloid hemangioma]]''; however, glomeruloid [[hemangioma]]s are not always associated with the POEMS syndrome.<ref name=pmid19077091>{{cite journal |author=González-Guerra E, Haro MR, Fariña MC, Martín L, Manzarbeitia L, Requena L |title=Glomeruloid haemangioma is not always associated with POEMS syndrome |journal=Clin. Exp. Dermatol. |volume=34 |issue=7 |pages=800–3 |year=2009 |month=October |pmid=19077091 |doi=10.1111/j.1365-2230.2008.02997.x |url=}}</ref> | *The [[pathologist]] may come across an undiagnosed case in the form of a ''[[glomeruloid hemangioma]]''; however, glomeruloid [[hemangioma]]s are not always associated with the POEMS syndrome.<ref name=pmid19077091>{{cite journal |author=González-Guerra E, Haro MR, Fariña MC, Martín L, Manzarbeitia L, Requena L |title=Glomeruloid haemangioma is not always associated with POEMS syndrome |journal=Clin. Exp. Dermatol. |volume=34 |issue=7 |pages=800–3 |year=2009 |month=October |pmid=19077091 |doi=10.1111/j.1365-2230.2008.02997.x |url=}}</ref> | ||
*POEMS syndrome typically cause osteosclerotic bone deposits (so-called osteosclerotic myeloma), in contrast to the lytic lesions seen in more conventional [[plasma cell myeloma]].{{fact}} | |||
==See also== | ==See also== | ||
*[[POEM]]. | *[[POEM]] - a treatment for [[achalasia]]. | ||
==References== | ==References== | ||