Difference between revisions of "POEMS syndrome"

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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 |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>
==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:''
''Mandatory:''
;1. Polyneuropathy (typically demyelinating)
# Polyneuropathy (typically demyelinating)
;2. Monoclonal plasma cell proliferation (usually lambda)
# Monoclonal plasma cell proliferation (usually lambda)
''Major:''
''Major:''
;3. [[Castleman disease]]
# [[Castleman disease]]
;4. Sclerotic bone lesions
# Sclerotic bone lesions
;5. Vascular endothelial growth factor elevation
# Vascular endothelial growth factor elevation
''Minor:''
''Minor:''
;6. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy)
# Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy)
;7. Extravascular volume overload
# Extravascular volume overload
;8. Endocrinopathy
# Endocrinopathy
;9. Sking changes
# Sking changes
;10. Papilloedema
# Papilloedema
;11. Thrombocytosis/polycythemia
# 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]].

Revision as of 17:33, 25 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. Sking changes
  5. Papilloedema
  6. 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.

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.
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