Difference between revisions of "POEMS syndrome"

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m
typically osteosclerotic
m (diagnostic criteria)
m (typically osteosclerotic)
 
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*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>
==Diagnostic criteria==
Mandatory:
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>
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]].
''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==
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