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POEMS Syndrome (poems + syndrome)
Selected AbstractsPolyneuropathy in POEMS syndromeEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 5 2008Khaled Ashawesh No abstract is available for this article. [source] Sural nerve and epidermal vascular abnormalities in a case of POEMS syndromeEUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2006L. Santoro First page of article [source] Solitary glomeruloid haemangioma without POEMS syndromeJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2005D. Vélez Background:, The term ,glomeruloid haemangioma' was coined by Chan et al. for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in patients without this syndrome. Methods:, We report on an 86-year-old man without POEMS syndrome and with a solitary red papule on the face. Results:, A cutaneous biopsy showed histological findings consistent with a glomeruloid haemangioma. Physical examination of the skin did not show any other cutaneous lesion and laboratory and radiological studies ruled out the presence of POEMS syndrome. Conclusions:, Glomeruloid haemangiomas could exceptionally be present as solitary vascular tumours and out of the context of POEMS syndrome. To our knowledge, this is the first case reported of glomeruloid haemangioma without POEMS syndrome. Moreover, the presentation on the face is also highly unusual. [source] A case of POEMS syndrome with high concentrations of interleukin-6 in pericardial fluidJOURNAL OF INTERNAL MEDICINE, Issue 2 2001N. Shikama Abstract. Shikama N, Isono A, Otsuka Y, Terano T, Hirai A (Department of Internal Medicine, Chiba Municipal Hospital; and Second Department of Internal Medicine, Chiba University School of Medicine, Chiba, Japan). A case of POEMS syndrome with high concentrations of interleukin-6 in pericardial fluid. J Intern Med 2001; 250: 170,173. The POEMS syndrome (plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy of various forms, monoclonal gammopathy, skin changes) is a rare multisystem disorder of unknown pathogenesis. Overexpression of proinflammatory cytokines has been implicated in the pathogenesis of POEMS syndrome, however, it is not known whether there is an association between abnormalities in cytokines and pericardial fluid. We present a case of POEMS syndrome with high concentrations of interleukin-6 (IL-6) in pericardial fluid. In our patient, pericarditis developed into cardiac tamponade, and the concentration of IL-6 in pericardial fluid was remarkably elevated compared with that in serum (1760 vs. 6.57 pg mL,1). We suggest that IL-6 is associated with the progression or maintenance of pericarditis as a result of POEMS syndrome. [source] Immunotherapy of idiopathic inflammatory neuropathiesMUSCLE AND NERVE, Issue 3 2003Peter D. Donofrio MD Abstract Evaluation of peripheral neuropathy is a common reason for referral to a neurologist. Recent advances in immunology have identified an inflammatory component in many neuropathies and have led to treatment trials using agents that attenuate this response. This article reviews the clinical presentation and treatment of the most common subacute inflammatory neuropathies, Guillain,Barré syndrome (GBS) and Fisher syndrome, and describes the lack of response to corticosteroids and the efficacy of treatment with plasma exchange and intravenous immunoglobulin (IVIG). Chronic inflammatory demyelinating polyneuropathy, although sharing some clinical, electrodiagnostic, and pathologic similarities to GBS, improves after treatment with plasma exchange and IVIG and numerous immunomodulatory agents. Controlled trials in multifocal motor neuropathy have shown benefit after treatment with IVIG and cyclophosphamide. Also discussed is the treatment of less common inflammatory neuropathies whose pathophysiology involves monoclonal proteins or antibodies directed against myelin-associated glycoprotein or sulfatide. Little treatment data exist to direct the clinician to proper management of rare inflammatory neuropathies resulting from osteosclerotic myeloma; POEMS syndrome; vasculitis; Sjögren's syndrome; and neoplasia (paraneoplastic neuropathy). Muscle Nerve 28: 273,292, 2003 [source] Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2010Trista Stankowski-Drengler POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) syndrome is a rare paraneoplastic syndrome in which nearly all patients have a monoclonal lambda restricted plasma cell disorder. We investigated whether patients with POEMS have abnormal serum immunoglobulin free light chain (FLC) ratios. Fifty patients with newly diagnosed POEMS syndrome were assessed. Cystatin C levels were measured to discern whether subclinical renal insufficiency could account for FLC elevations in the presence of a normal FLC ratio. Forty-five patients (90%) had elevated lambda FLC; however, only nine (18%) had abnormal FLC ratios. The rise in serum FLC of POEMS patients appeared to be multifactorial,both a function of subclinical renal insufficiency and polyclonal activation of medullary and extramedullary plasma cells. Those patients expressing a clonal IgA were more likely to have clonal plasmacytosis observed on iliac crest biopsy than those with IgG. In summary, serum immunoglobulin profiles are unique in POEMS syndrome as compared with other plasma cell disorders. Am. J. Hematol. 2010. © 2010 Wiley-Liss, Inc. [source] Coexistence of primary AL amyloidosis and POEMS syndrome: Efficacy of melphalan-dexamethasone and role of biochemical markers in monitoring the diseases course,AMERICAN JOURNAL OF HEMATOLOGY, Issue 2 2010Fausto Adami No abstract is available for this article. [source] | ||||||||||||||||||||||