POEMS syndrome

POEMS syndrome is a paraneoplastic syndrome characterized by polyradiculoneuropathy (P), organomegaly (O), endocrinopathy (E), clonal plasma cell disorder (M), and skin changes (S). Other features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis, and elevated VEGF levels.

Paraproteinemia

An abnormal immunoglobulin or part of an Ig (light chain) in the circulation. Paraproteins are typically produced by a clonal population of B-cell derived plasma cells.


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PMID (PMCID)
26663802
 MALE Middle Aged
POEMS Syndrome with IgG-lamda/IgA-kappa Biclonal Gammopathy and Abnormal Serum Free Light Chain Ratio: a Case Report.
Ham JY, Suh JS, Lee WK, Song KE.
Ann Clin Lab Sci. 2015;45(6):702-6.
However, a primary pathogenic role of the different paraproteinemia in POEMS syndrome remains unclear.
18342713
 MALE
IgM-lambda paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome.
Colaco SM, Miller T, Ruben BS, Fogarty PF, Fox LP.
J Am Acad Dermatol. 2008;58(4):671-5.
IgM-lambda paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome.
1437299
 MALE Middle Aged
Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia.
Romas E, Storey E, Ayers M, Byrne E.
Pathology. 1992;24(3):217-20.
Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change (POEMS) syndrome with IgG kappa paraproteinemia.
2157280
 MALE Middle Aged
[Peripheral neuropathy with monoclonal gammopathy].
Meier T, Meyer M.
Schweiz Med Wochenschr. 1990;120(12):417-25.
Demyelinating PN with IgG-lambda- or IgA-lambda paraproteinemia occurs often in the uncommon but readily treatable osteosclerotic myeloma.