Difference between revisions of "POEMS syndrome"
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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 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> | ||
Mandatory: | ''Mandatory:'' | ||
1. Polyneuropathy (typically demyelinating) | ;1. Polyneuropathy (typically demyelinating) | ||
2. Monoclonal plasma cell proliferation (usually lambda) | ;2. Monoclonal plasma cell proliferation (usually lambda) | ||
Major: | ''Major:'' | ||
3. [[Castleman disease]] | ;3. [[Castleman disease]] | ||
4. Sclerotic bone lesions | ;4. Sclerotic bone lesions | ||
5. Vascular endothelial growth factor elevation | ;5. Vascular endothelial growth factor elevation | ||
Minor: | ''Minor:'' | ||
6. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy) | ;6. Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy) | ||
7. Extravascular volume overload | ;7. Extravascular volume overload | ||
8. Endocrinopathy | ;8. Endocrinopathy | ||
9. Sking changes | ;9. Sking changes | ||
10. Papilloedema | ;10. Papilloedema | ||
11. Thrombocytosis/polycythemia | ;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]]. | 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 14:14, 26 July 2020
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:
- 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.
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]
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.
- ↑ "POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management". Am. J. Hematol. 94 (7): 812–827. July 2019. 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.