Difference between revisions of "POEMS syndrome"

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m (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.
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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 |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==

Latest revision as of 22:34, 26 March 2021

POEMS syndrome is a constellation of findings, best thought of as a paraneoplastic syndrome due to clonal plasma cells:[1]

  • Polyneuropathy.
  • Organomegaly.
  • Endocrinopathy.
  • M-protein.
  • Skin changes.

Diagnostic criteria

Requires both mandatory criteria and at least one each from major and minor criteria:[2]

Mandatory:

  1. Polyneuropathy (typically demyelinating).
  2. Monoclonal plasma cell proliferation (usually lambda).

Major:

  1. Castleman disease
  2. Sclerotic bone lesions.
  3. Vascular endothelial growth factor elevation.

Minor:

  1. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy).
  2. Extravascular volume overload.
  3. Endocrinopathy.
  4. Skin changes.
  5. Papilloedema.
  6. Thrombocytosis/polycythemia.

Pathology

See also

References

  1. Yuri T, Yamazaki F, Takasu K, Shikata N, Tsubura A (June 2008). "Glomeruloid hemangioma". Pathol. Int. 58 (6): 390–5. doi:10.1111/j.1440-1827.2008.02241.x. PMID 18477219.
  2. Dispenzieri A (July 2019). "POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management". Am. J. Hematol. 94 (7): 812–827. doi:10.1002/ajh.25495. PMID 31012139.
  3. González-Guerra E, Haro MR, Fariña MC, Martín L, Manzarbeitia L, Requena L (October 2009). "Glomeruloid haemangioma is not always associated with POEMS syndrome". Clin. Exp. Dermatol. 34 (7): 800–3. doi:10.1111/j.1365-2230.2008.02997.x. PMID 19077091.