Disease relevance of POEMS Syndrome

• The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood [1].
• To evaluate a possible implication of cytokines in the pathogenesis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, we studied five consecutive patients with this condition, of which four had sclerotic bone lesions and four had multicentric Castleman's disease [2].
• Steroid treatment resulted in complete disappearance of the biliverdinaemia in this patient as well as symptoms and signs of POEMS syndrome, suggesting that the biliverdinaemia was related to POEMS syndrome [3].
• We suggest that IL-6 is associated with the progression or maintenance of pericarditis as a result of POEMS syndrome [4].
• Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome [5].

High impact information on POEMS Syndrome

• Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome [6].
• Hypoparathyroidism in POEMS syndrome [7].
• IL-1ra and sTNFrs were increased in POEMS syndrome, but out of proportion to the increase of IL-1 beta and TNF-alpha [8].
• Elevated serum levels of IL-1 beta (13/13 samples), and IL-6 (7/13 samples) were found in patients with POEMS syndrome [2].
• We conclude that IL-1 beta and IL-6 serum levels may be chronically elevated in patients with POEMS syndrome, and that lymph node may be one site of IL-1 beta overproduction [2].

Chemical compound and disease context of POEMS Syndrome

• All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflammatory cytokines [9].
• Improvement in the POEMS syndrome after administration of tamoxifen [10].
• A patient with severe widespread POEMS syndrome resistant to melphalan who experienced a dramatic improvement after high-dose melphalan followed by autologous haematopoietic cell transplantation (AHCT) is reported [11].
• Thalidomide in POEMS syndrome: case report [12].
• Glucocorticoid therapy may be beneficial in Crow-Fukase syndrome to improve the distorted metabolism of DHEA with no adverse effect on the diabetes [13].

Biological context of POEMS Syndrome

• Predictive value of serial platelet count and VEGF determination for the management of DIC in the Crow-Fukase (POEMS) syndrome [14].
• The first category includes cases displaying regular UML, which was observed in 43 cases; it was more frequent in 9 cases with polyneuropathy organomegaly endocrinopathy m-protein skin changes (POEMS) syndrome as well as in 1 case of Charcot-Marie-Tooth 1B with a novel point mutation in the P0 gene [15].

Anatomical context of POEMS Syndrome

• Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature [16].
• We describe a patient with POEMS syndrome and SLE and review the similarities and differences between POEMS syndrome and other connective tissue disorders [17].
• The authors describe a patient with POEMS syndrome who has polyneuropathy of the upper and lower extremities, splenomegaly, impotence, IgA-lambda monoclonal protein (M-protein), and marked thickening of his skin [18].
• The diagnosis of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein and Skin changes was suspected clinically and confirmed with a bone marrow biopsy [19].
• An autopsy case of POEMS syndrome with a high level of IL-6 and VEGF in the serum and ascitic fluid [20].

Gene context of POEMS Syndrome

• A pathogenic role for VEGF in POEMS syndrome appears to be likely, but its causal relation to the nephropathy awaits further investigation [21].
• Proinflammatory cytokines (TNF-alpha, IL-1, IL-6) have been implicated in the physiopathogenesis of POEMS syndrome, particularly when there is renal involvement [22].
• Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 are increased in POEMS syndrome [23].
• The patient's multiple clinical manifestations, including cardiomyopathy, improved with chemotherapy, which is characteristic of POEMS syndrome [24].
• POEMS syndrome in systemic lupus erythematosus [17].

Analytical, diagnostic and therapeutic context of POEMS Syndrome

• Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome [25].

References