POEMS syndrome , also known as osteosclerotic myeloma, Takatsuki syndrome , and Crow–Fukase syndrome [3, 4], is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The acronym POEMS refers to several, but not all, of the features of the syndrome: polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Not all of the features within the acronym are required to make the diagnosis. There are other important features not included in the POEMS acronym, including papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis/erythrocytosis (P.E.S.T.), elevated vascular endothelial growth factor (VEGF) levels, abnormal pulmonary function tests, and a predisposition towards thrombosis. Lastly, there is a Castleman’s disease variant of POEMS syndrome that may not be associated with a clonal plasma cell disorder [5, 6]. Table 15.1 outlines the range of expected frequencies of each of the features based on the largest published series [2, 7–11]. VEGF is the cytokine that correlates best with disease activity [12–20], although it may not be the driving force of the disease based on the mixed results seen with anti-VEGF therapy [5, 21–29]. The pathogenesis of the syndrome is not well understood. VEGF, which is expressed by osteoblasts, macrophages, tumor cells  (including plasma cells) [31, 32], and megakaryocytes/platelets , is known to target endothelial cells, induce a rapid and reversible increase in vascular permeability, and be important in angiogenesis. Both IL-1β and IL-6 have been shown to stimulate VEGF production . Interleukin 12 has also shown to be quite elevated in patients with POEMS syndrome . Little is known about the plasma cells in POEMS syndrome except that more than 95 % of the time they are lambda light chain-restricted with restricted immunoglobulin light chain variable gene usage (IGLV1) .
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