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https://www.readbyqxmd.com/read/28653516/-refractory-sweet-syndrome-complicated-by-monoclonal-gammopathy-of-undetermined-significance
#1
I Kechaou, E Cherif, I Boukhris, S Azzabi, L Ben Hassine
The association between Sweet syndrome and monoclonal gammopathy of undetermined significance (MGUS) is exceptional. We report the case of a 44 years-old woman in whom recurrent Sweet syndrome was complicated by monoclonal gammopathy of undetermined significance (MGUS) after 3 years of evolution.
2017: Revue Médicale de Bruxelles
https://www.readbyqxmd.com/read/28645246/igmk-paraprotein-from-gammopathy-patient-can-bind-to-cardiolipin-and-interfere-with-coagulation-assay-a-case-report
#2
Xin-Yao Wu, Yu-Feng Yin, Jia-Lin Teng, Li-Wei Zhang, Cheng-de Yang
BACKGROUND: The monoclonal gammopathies are a group of plasma-cell proliferative disorders characterized by the secretion of monoclonal immunoglobulin (M protein or paraprotein). Some rare cases have revealed the specific affinity of paraprotein as autoantibody. Here we report a patient with monoclonal gammopathy of undetermined significance (MGUS) accompanied by a remarkable increase of anticardiolipin antibody (aCL) and an extensively decreased coagulation factor activity, however, without any clinical signs of antiphospholipid syndrome (APS) and bleeding...
June 23, 2017: BMC Immunology
https://www.readbyqxmd.com/read/28602036/circulating-adiponectin-levels-differ-between-patients-with-multiple-myeloma-and-its-precursor-disease
#3
Jonathan N Hofmann, Sham Mailankody, Neha Korde, Ye Wang, Nishant Tageja, Rene Costello, Adriana Zingone, Malin Hultcrantz, Michael N Pollak, Mark P Purdue, Ola Landgren
OBJECTIVE: An increased risk of multiple myeloma (MM) has been observed among individuals with low prediagnostic circulating levels of adiponectin, a metabolic hormone that is typically underexpressed among those with overweight or obesity. To assess whether adiponectin may influence myeloma development or progression to frank MM, circulating adiponectin levels were compared across patients with different stages of MM and its precursor, monoclonal gammopathy of undetermined significance (MGUS)...
June 11, 2017: Obesity
https://www.readbyqxmd.com/read/28550183/somatic-mutation-spectrum-in-monoclonal-gammopathy-of-undetermined-significance-indicates-a-less-complex-genomic-landscape-compared-to-multiple-myeloma
#4
Aneta Mikulasova, Christopher P Wardell, Alexander Murison, Eileen M Boyle, Graham H Jackson, Jan Smetana, Zuzana Kufova, Ludek Pour, Viera Sandecka, Martina Almasi, Pavla Vsianska, Evzen Gregora, Petr Kuglik, Roman Hajek, Faith E Davies, Gareth J Morgan, Brian A Walker
Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant precursor of multiple myeloma with a 1% risk of progression per year. Although targeted analyses have shown the presence of specific genetic abnormalities such as IGH translocations, RB1 deletion, 1q gain, hyperdiploidy or RAS genes mutations, little is known about the molecular mechanism of malignant transformation. We have performed whole exome sequencing together with CGH+SNP array analysis in 33 flow-cytometry separated abnormal plasma cell samples from MGUS patients to describe somatic gene mutations and chromosome changes at the genome-wide level...
May 26, 2017: Haematologica
https://www.readbyqxmd.com/read/28540748/expression-of-the-il-6-receptor-alpha-chain-cd126-in-normal-and-abnormal-plasma-cells-in-monoclonal-gammopathy-of-undetermined-significance-and-smoldering-myeloma
#5
Dalia Abdel-Raouf Salem, Neha Korde, David J Venzon, David J Liewehr, Irina Maric, Katherine R Calvo, Raul Braylan, Prashant R Tembhare, Constance M Yuan, Carl Ola Landgren, Maryalice Stetler-Stevenson
IL-6 activity in normal plasma cells (nPCs) and abnormal plasma cells (aPCs) is CD126 (subunit of IL-6 receptor) dependent. We quantified CD126 expression on nPCs and aPCs in monoclonal gammopathy of undetermined significance (MGUS), smoldering myeloma (SMM), and multiple myeloma (MM). CD126 was detected on all nPCs and aPCs indicating that CD126 does not have diagnostic utility. CD126 expression was higher in aPCs than in nPCs in 85% SMM but only 41% MGUS and there was evidence that CD126 was higher in aPCs than nPCs in the SMM (p = ...
May 25, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28512563/acquired-von-willebrand-syndrome-associated-to-secondary-igm-mgus-emerging-after-autologous-stem-cell-transplantation-for-al-amyloidosis
#6
Hina Qamar, Adrienne Lee, Karen Valentine, Leslie Skeith, Victor H Jimenez-Zepeda
Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28508985/light-chain-fanconi-syndrome-in-a-patient-with-acute-myeloid-leukemia-and-monoclonal-gammopathy-of-undetermined-significance
#7
Daniel W Ross, Rimda Wanchoo, Adriana Guigova, Cristina Ghiuzeli, Steven L Allen, Kenar D Jhaveri
Proximal tubules are a target for paraproteinemic diseases. Cast nephropathy, light chain deposition diseases, and amyloidosis are frequently encountered in patients with multiple myeloma. Rarely, a subset of patients develop light chain Fanconi syndrome (LCFS). LCFS has been reported with multiple myeloma, monoclonal gammopathy of renal significance (MGRS), chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia and diffuse large B-cell lymphoma. No cases have been described with other hematologic malignancies...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508971/short-term-outcome-and-quality-of-life-in-kidney-transplant-recipient-with-monoclonal-gammopathy
#8
Masaaki Yanishi, Hiroyasu Tsukaguchi, Takashi Yoshida, Hisanori Taniguchi, Kenji Yoshida, Takao Mishima, Yoshihiro Komai, Kaneki Yasuda, Masato Watanabe, Motohiko Sugi, Hidefumi Kinoshita, Tadashi Matsuda
Monoclonal gammopathy of undetermined significance (MGUS) is the common pre-malignant B cell disorders with a general prevalence of 3-5 % at age over 50. Because of the potential malignant transformation and immune insufficiency, pre-transplant MGUS recipient should be carefully followed after allograft transplantation. The post-transplant prognosis and quality of life (QOL) in patient with MGUS have not yet been fully determined. The aim of this study is to evaluate function and pathology of the renal allograft and self-assessment QOL changes during 2 years after transplantation in our case of MGUS-bearing recipient...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28502031/vitamin-d-and-plasma-cell-dyscrasias-reviewing-the-significance
#9
REVIEW
Nicholas Burwick
Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder and precursor disease to multiple myeloma and other related cancers. While MGUS is considered a benign disorder, with a low risk of disease progression, patients have altered bone microarchitecture and an increased risk of bone fracture. In addition, alterations in immune function are regularly found to correlate with disease activity. Vitamin D, an important hormone for bone and immune health, is commonly deficient in multiple myeloma patients...
May 13, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28493835/expression-and-release-of-glucose-regulated-protein-78-grp78-in-multiple-myeloma
#10
Normann Steiner, Bojana Borjan, Roman Hajek, Karin Jöhrer, Georg Göbel, Wolfgang Willenbacher, Johann Kern, Eberhard Gunsilius, Gerold Untergasser
INTRODUCTION: Multiple myeloma (MM) is a plasma cell neoplasm that is mostly incurable due to acquired resistance during the treatment course. Thus, we evaluated expression and release of glucose-regulated protein 78 kDa (GRP78/BiP), an endoplasmic reticulum (ER) based pro-survival chaperone involved in immunoglobulin folding and unfolded protein responses. RESULTS: GRP78 protein expression in the ER and on the cell surface did not significantly differ between MGUS, NDMM and RRMM patients although there was a trend to higher surface expression in RRMM...
April 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28490364/sporadic-late-onset-nemaline-myopathy-clinico-pathological-characteristics-and-review-of-76-cases
#11
REVIEW
Lukas J Schnitzler, Tobias Schreckenbach, Aleksandra Nadaj-Pakleza, Werner Stenzel, Elisabeth J Rushing, Philip Van Damme, Andreas Ferbert, Susanne Petri, Christian Hartmann, Antje Bornemann, Andreas Meisel, Jens A Petersen, Thomas Tousseyn, Dietmar R Thal, Jens Reimann, Peter De Jonghe, Jean-Jacques Martin, Peter Y Van den Bergh, Jörg B Schulz, Joachim Weis, Kristl G Claeys
BACKGROUND: Sporadic late-onset nemaline myopathy (SLONM) is a rare, late-onset muscle disorder, characterized by the presence of nemaline rods in muscle fibers. Phenotypic characterization in a large cohort and a comprehensive overview of SLONM are lacking. METHODS: We studied the clinico-pathological features, treatment and outcome in a large cohort of 76 patients with SLONM, comprising 10 new patients and 66 cases derived from a literature meta-analysis (PubMed, 1966-2016), and compared these with 15 reported HIV-associated nemaline myopathy (HIV-NM) cases...
May 11, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28482711/the-presence-of-monoclonal-gammopathy-in-ph-negative-myeloproliferative-neoplasms-is-associated-with-a-detrimental-effect-on-outcomes
#12
Lenaïg Le Clech, Mehdi Sakka, Ahmed Meskar, Helene Kerspern, Jean-Richard Eveillard, Christian Berthou, Caroline Buors, Eric Lippert, Gaelle Guillerm, Isabelle Quintin-Roué, Jean-Luc Carré, Jean-Christophe Ianotto
Many case reports have indicated the occurrence of monoclonal gammopathy of uncertain significance (MGUS) or multiple myeloma (MM) in patients with Ph-negative myeloproliferative neoplasms (MPN), but few cohorts of patients have been published. This study concerns 667 patients newly diagnosed with polycythemia vera (PV) or essential thrombocythemia (ET) who were tested for monoclonal (M) protein at diagnosis (13.9% of patients). The overall survival of patients with M protein was dramatically lower than that of patients without M protein (12...
May 9, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28479151/laboratory-testing-for-monoclonal-gammopathies-focus-on-monoclonal-gammopathy-of-undetermined-significance-and-smoldering-multiple-myeloma
#13
REVIEW
Maria A V Willrich, David L Murray, Robert A Kyle
Monoclonal gammopathies (MG) are defined by increased proliferation of clonal plasma cells, resulting in a detectable abnormality called monoclonal component or M-protein. Detection of the M-protein as either narrow peaks on protein electrophoresis and discrete bands on immunofixation is the defining feature of MG. MG are classified as low-tumor burden disorders, pre-malignancies and malignancies. Since significant disease can be present at any level, several different tests are employed in order to encompass the inherent diverse nature of the M-proteins...
May 4, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28476134/overexpression-of-rkip-and-its-cross-talk-with-several-regulatory-gene-products-in-multiple-myeloma
#14
REVIEW
Anna Shvartsur, Kevin B Givechian, Hermes Garban, Benjamin Bonavida
Multiple myeloma (MM) is a clonal plasma-cell neoplastic disorder arising from an indolent premalignant disease known as monoclonal gammopathy of undetermined significance (MGUS). MM is a biologically complex heterogeneous disease reflected by its variable clinical responses of patients receiving the same treatment. Therefore, a molecular identification of stage-specific biomarkers will support a more individualized precise diagnostic/prognostic approach, an effective therapeutic regime, and will assist in the identification of novel therapeutic molecular targets...
May 5, 2017: Journal of Experimental & Clinical Cancer Research: CR
https://www.readbyqxmd.com/read/28472865/electrophysiological-features-of-poems-syndrome-compared-to-mgus-related-neuropathy
#15
Hyunjin Kim, Young-Min Lim, Joo Yea Jin, Dok Hyun Yoon, Cheolwon Suh, Sun-Young Kim, Jae-Cheol Jo, Kwang-Kuk Kim
INTRODUCTION: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and monoclonal gammopathy of undetermined significance (MGUS) are paraproteinemic disorders that can cause demyelinating polyneuropathy. Here, we assessed the findings of nerve conduction studies (NCSs) in patients with POEMS syndrome and MGUS-related neuropathy to determine whether the NCS characteristics can help differentiate between the conditions. METHODS: We enrolled 24 POEMS and 37 MGUS-related neuropathy patients...
May 4, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28467313/igg-kappa-monoclonal-gammopathy-of-unknown-significance-with-al-amyloidosis-simulating-giant-cell-arteritis
#16
V M Pompilian, S Tanaseanu, Camelia Badea, Sabina Zurac, C Socoliuc, Sorina Badelita, Elena Botez, Mihaela Antohe
Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA).We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension...
May 3, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28466550/cutting-edge-genomics-reveal-new-insights-into-tumour-development-disease-progression-and-therapeutic-impacts-in-multiple-myeloma
#17
REVIEW
Ankit K Dutta, Duncan R Hewett, J Lynn Fink, John P Grady, Andrew C W Zannettino
Multiple Myeloma (MM) is a haematological malignancy characterised by the clonal expansion of plasma cells (PCs) within the bone marrow. Despite advances in therapy, MM remains a largely incurable disease with a median survival of 6 years. In almost all cases, the development of MM is preceded by the benign PC condition Monoclonal Gammopathy of Undetermined Significance (MGUS). Recent studies show that the transformation of MGUS to MM is associated with complex genetic changes. Understanding how these changes contribute to evolution will present targets for clinical intervention...
May 3, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28456791/nk-cells-and-multiple-myeloma-associated-endothelial-cells-molecular-interactions-and-influence-of-il-27
#18
Alessandra Dondero, Beatrice Casu, Francesca Bellora, Angelo Vacca, Annunziata De Luisi, Maria Antonia Frassanito, Claudia Cantoni, Silvia Gaggero, Daniel Olive, Alessandro Moretta, Cristina Bottino, Roberta Castriconi
Angiogenesis represents a hallmark of tumor progression in Multiple Myeloma (MM), a still incurable malignancy. Here we analyzed the activity of cytokine-stimulated NK cells against tumor-associated endothelial cells isolated from bone marrow aspirates of MM patients with active disease (MMECs). We show that NK cells activated with optimal doses of IL-15 killed MMECs thanks to the concerted action of multiple activating receptors. In particular, according to the high expression of PVR and Nectin-2 on MMECs, DNAM-1 actively participated in target recognition...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28439985/the-utility-of-mass-fix-to-detect-and-monitor-monoclonal-proteins-in-the-clinic
#19
Paolo Milani, David L Murray, David R Barnidge, Mindy C Kohlhagen, John R Mills, Giampaolo Merlini, Surendra Dasari, Angela Dispenzieri
The detection and quantification of monoclonal-proteins (M-proteins) are necessary for the diagnosis and evaluation of response in plasma cell dyscrasias. Immunoglobulin enrichment-coupled with matrix-assisted laser desorption ionization time-of-flight mass-spectrometry (MASS-FIX) is a simple and inexpensive method to identify M-proteins, but its clinical generalizability has not yet been elucidated. We compared MASS-FIX to protein electrophoresis (PEL), serum/urine immunofixation-electrophoresis (IFE) and quantitative serum free-light chain (FLC) for the identification of M-proteins in different clinical diagnoses...
April 25, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28425076/monoclonal-gammopathy-of-renal-significance-mgrs-the-characteristics-and-significance-of-a-new-meta-entity
#20
REVIEW
Mariana Ciocchini, Jorge Arbelbide, Carlos G Musso
Monoclonal gammopathy of renal significance (MGRS) is a new nosological group of entities (meta-entity) defined in 2012, whose pathogenesis depends on monoclonal immunoglobulins (Ig) secreted by low-grade lymphoproliferative disorders, which belong to M-protein-related diseases. Renal damage is the result of monoclonal Ig deposit or its activity as autoantibodies, which can compromise any nephronal area. MGRS does not include kidney diseases produced by high-grade lymphoproliferative disorders as well as those whose pathogenesis are independent of monoclonal Ig (such as drug toxicity or metabolic disorders)...
April 19, 2017: International Urology and Nephrology
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