POEMS syndrome

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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

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. "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.
  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.