<unk><unk>  is an acronym, where the 'P' stands for polyneuropathy, 'O' for organomegaly (specifically of the liver and spleen), 'E' for endocrinopathy, 'M' for immunoglobulin (para-protein or M-protein) and 'S' for skin changes.<unk><unk>  is a para-neoplastic<unk>  characterized by multi-organ system dysfunction, monoclonal plasma cell proliferation and demyelinating inflammatory polyneuropathy. The lambda component of immunoglobulin light chains is thought to be overproduced in<unk><unk>  secondary to monoclonal plasma cell proliferation. Bone marrow studies of patients suffering from<unk><unk>  shows lymphoid aggregates rimmed by monotypic or polytypic plasma cells. The monoclonal gammopathy or overproduction of lambda light chain stimulates the monocyte/macrophage system to produce various proinflammatory cytokines. such as interleukin-1, interleukin-6, interleukin-12, vascular endothelial growth factor (VEGF) and TNF-Alpha. Transforming growth factor beta (TGF-beta), an inhbitory cytokine is thought to be underproduced in patients suffering from<unk><unk>  leading to a storm of proinflammatory cytokines which drive the disease process. The overproduction of this IL-6 may be related to some of the clinical features seen in<unk><unk>  such as gammopathy, elevated C-Reactive protein (CRP) and thrombocytosis. The mesangial proliferation and membranoproliferative glomerulonephritis (MPGN) seen in<unk><unk>  is thought to be secondary to increased production of platelet-derived growth factor (PDGF) and fibroblast growth factor beta (FGF-β) have been implicated in mesangial cell proliferation. The neovascularization induced by VEGF leads to increased vascular permeability. Since the increased production of VEGF is unchecked due to the plasma cell dyscrasia, the consequence is an unregulated extravasation of intravascular fluid into the extravascular compartment. resulting in edema.<unk><unk>  is known to be associated multiple myeloma (osteosclerotic type), Castleman's disease, plasmacytoma, monoclonal gammopathy of undetermined significance (MGUS), pulmonary hypertension, restrictive lung disease, thrombophilia, cardiac amyloidosis and papilledema.
POEMS syndrome pathophysiology
Primary amyloidosis pathophysiology
Familial amyloidosis pathophysiology
Multiple myeloma pathophysiology
Waldenström's macroglobulinemia pathophysiology
POEMS syndrome epidemiology and demographics
POEMS syndrome other imaging findings
POEMS syndrome other diagnostic studies