Difference between revisions of "POEMS syndrome"
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''Mandatory:'' | ''Mandatory:'' | ||
# Polyneuropathy (typically demyelinating) | # Polyneuropathy (typically demyelinating). | ||
# Monoclonal plasma cell proliferation (usually lambda) | # Monoclonal plasma cell proliferation (usually lambda). | ||
''Major:'' | ''Major:'' | ||
# [[Castleman disease]] | # [[Castleman disease]] | ||
# Sclerotic bone lesions | # Sclerotic bone lesions. | ||
# Vascular endothelial growth factor elevation | # Vascular endothelial growth factor elevation. | ||
''Minor:'' | ''Minor:'' | ||
# Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy) | # Organomegaly (splenomegaly, hepatomegaly or lymphadenopathy). | ||
# Extravascular volume overload | # Extravascular volume overload. | ||
# Endocrinopathy | # Endocrinopathy. | ||
# | # Skin changes. | ||
# Papilloedema | # Papilloedema. | ||
# 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 17:34, 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:
- 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.
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.
- ↑ 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.