Difference between revisions of "POEMS syndrome"
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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 '' | *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== | |||
*[[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:
- 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
- The pathologist may come across an undiagnosed case in the form of a glomeruloid hemangioma; however, glomeruloid hemangiomas are not always associated with the POEMS syndrome.[3]
- POEMS syndrome typically cause osteosclerotic bone deposits (so-called osteosclerotic myeloma), in contrast to the lytic lesions seen in more conventional plasma cell myeloma.[citation needed]
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 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.