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Monoclonal gammopathy

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https://www.readbyqxmd.com/read/28444965/serum-free-light-chain-quantitative-assays-dilemma-of-a-biomarker
#1
Giovanni Cigliana, Francesca Gulli, Cecilia Napodano, Krizia Pocino, Elena De Santis, Luigi Colacicco, Iole Cordone, Laura Conti, Umberto Basile
BACKGROUND: Serum free light chains detection assays are consistently meeting greater interest for the diagnosis and monitoring of monoclonal gammopathies and plasma cell dyscrasias. Nowadays, there are neither standardized methods nor reference material for the determination of free light chains; for this reason, it is important to compare two different assays used in clinical laboratory. METHODS: We evaluated 300 serum samples from patients with B-cell disorders and compared the analytical performances of both assay...
April 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28439995/scleromyxoedema-in-a-dog
#2
Aurore F Laprais, Petra Bizikova, Erin W Lashnits, Alison Tucker, Keith E Linder
BACKGROUND: In humans, scleromyxoedema is a chronic progressive skin condition traditionally characterized by deposits of mucin, increased number of fibroblasts and fibrosis in the skin, and by systemic disease. Thyroid disease is typically absent. A monoclonal gammopathy is usually present, as are other comorbidities. Descriptions of scleromyxoedema in the veterinary literature are limited to a single feline case. One dog, previously reported as having papular mucinosis, exhibited features that matched the more current diagnostic criteria of scleromyxoedema...
April 24, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28439875/epigenetic-repression-of-mir-375-is-the-dominant-mechanism-for-constitutive-activation-of-the-pdpk1-rps6ka3-signalling-axis-in-multiple-myeloma
#3
Shotaro Tatekawa, Yoshiaki Chinen, Masaki Ri, Tomoko Narita, Yuji Shimura, Yayoi Matsumura-Kimoto, Taku Tsukamoto, Tsutomu Kobayashi, Eri Kawata, Nobuhiko Uoshima, Tomohiko Taki, Masafumi Taniwaki, Hiroshi Handa, Shinsuke Iida, Junya Kuroda
Cytogenetic/molecular heterogeneity is the hallmark of multiple myeloma (MM). However, we recently showed that the serine/threonine kinase PDPK1 and its substrate RPS6KA3 (also termed RSK2) are universally active in MM, and play pivotal roles in myeloma pathophysiology. In this study, we assessed involvement of aberrant miR-375 repression in PDPK1 overexpression in MM. An analysis of plasma cells from 30 pre-malignant monoclonal gammopathies of undetermined significance and 73 MM patients showed a significant decrease in miR-375 expression in patient-derived plasma cells regardless of the clinical stage, compared to normal plasma cells...
April 25, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28425076/monoclonal-gammopathy-of-renal-significance-mgrs-the-characteristics-and-significance-of-a-new-meta-entity
#4
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
https://www.readbyqxmd.com/read/28422865/crystalglobulinemia-manifesting-as-chronic-arthralgia-and-acute-limb-ischemia-a-clinical-case-report
#5
Nobuya Abe, Tomoko Tomita, Miyuki Bohgaki, Hideki Kasahara, Takao Koike
RATIONALE: Crystalglobulinemia is a rare disease caused by monoclonal immunoglobulins, characterized by irreversible crystallization on refrigeration. It causes systemic symptoms including purpura, arthralgia, and vessel occlusive conditions to be exacerbated by exposure to cold. We report a patient with crystalglobulinemia associated with monoclonal gammopathy of undetermined significance (MGUS) manifesting as chronic arthralgia and recurrent acute arterial occlusion. PRESENTING CONCERNS: A 61-year-old man, who had been diagnosed with MGUS and who had arthralgia of unknown origin, presented with recurrent acute limb ischemia after surgical thromboembolectomy...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28417905/analytical-criticalities-associated-to-different-immunological-methods-for-serum-free-light-chain-detection-in-plasma-cell-dyscrasias-a-description-of-particular-clinical-cases
#6
Rocco Sabatino, Antonio Perrone, Marco Cuomo, Sandra Liotti, Vittoria Barchiesi, Monica Cantile, Ernesta Cavalcanti
Current criteria for differential diagnosis of multiple myeloma (MM), Monoclonal gammopathy of undetermined significance (MGUS), and smoldering multiple myeloma (SMM) are included in the 2003 guidelines by the International Myeloma Working Group (IMWG). An updated version was then published in 2014, highlighting the importance of serum free light chain (sFLC) detection, as well as the κ/λ ratio as excellent indicators of clonality. At present, two commercial assays for sFLC quantification are available: the Freelite™ assay and the N-Latex assay...
April 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28416003/characterization-and-risk-estimate-of-cancer-in-patients-with-primary-sj%C3%A3-gren-syndrome
#7
Pilar Brito-Zerón, Belchin Kostov, Guadalupe Fraile, Daniel Caravia-Durán, Brenda Maure, Francisco-Javier Rascón, Mónica Zamora, Arnau Casanovas, Miguel Lopez-Dupla, Mar Ripoll, Blanca Pinilla, Eva Fonseca, Miriam Akasbi, Gloria de la Red, Miguel-Angel Duarte-Millán, Patricia Fanlo, Pablo Guisado-Vasco, Roberto Pérez-Alvarez, Antonio J Chamorro, César Morcillo, Iratxe Jiménez-Heredia, Isabel Sánchez-Berná, Armando López-Guillermo, Manuel Ramos-Casals
BACKGROUND: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS). METHODS: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender...
April 17, 2017: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/28415715/epha3-acts-as-proangiogenic-factor-in-multiple-myeloma
#8
Antonella Caivano, Francesco La Rocca, Ilaria Laurenzana, Tiziana Annese, Roberto Tamma, Ubaldo Famigliari, Vittorio Simeon, Stefania Trino, Luciana De Luca, Oreste Villani, Simona Berardi, Antonio Basile, Angelo Vacca, Giuseppe Saglio, Luigi Del Vecchio, Pellegrino Musto, Daniela Cilloni
This study investigates the role of ephrin receptor A3 (EphA3) in the angiogenesis of Multiple Myeloma (MM) and the effects of a selective target of EphA3 by a specific monoclonal antibody on primary bone marrow endothelial cells (ECs) of MM patients.EphA3 mRNA and protein were evaluated in ECs of MM patients (MMECs), in ECs of patients with monoclonal gammopathies of undetermined significance (MGECs) and in ECs of healthy subjects (control ECs). The effects of EphA3 targeting by mRNA silencing (siRNA) or by the anti EphA3 antibody on the angiogenesis were evaluated...
March 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28412476/monoclonal-gammopathy-of-undetermined-significance-and-biological-treatments-what-should-we-do
#9
EDITORIAL
Émilie Lemieux-Blanchard
No abstract text is available yet for this article.
April 12, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28405536/an-unfortunate-polyneuropathy-organomegaly-endocrinopathy-monoclonal-gammopathy-and-skin-change-poems
#10
Faraz Afridi, Jorge Otoya, Samantha F Bunting, Gerard Chaaya
POEMS syndrome is an acronym for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, which is a rare paraneoplastic disease of monoclonal plasma cells. A mandatory criterion to diagnose POEMS syndrome is the presence of a monoclonal plasma cell dyscrasia in which plasma cell leukemia is the most aggressive form. Early identification of the features of the POEMS syndrome is critical for patients to identify an underlying plasma cell dyscrasias and to reduce the morbidity and mortality of the disease by providing early therapy...
March 8, 2017: Curēus
https://www.readbyqxmd.com/read/28403522/structure-activity-relationship-studies-spr-affinity-characterization-and-conformational-analysisof-peptides-mimicking-the-hnk-1-carbohydrate-epitope
#11
Paolo Rovero, Matthaia Ieronymaki, Francesca Nuti, Diego Brancaccio, Giada Rossi, Feliciana Real-Fernández, Yihong Cao, Olivier Monasson, Maud Larregola, Elisa Peroni, Jacques Uziel, Giuseppina Sabatino, Ettore Novellino, Alfonso Carotenuto, Anna Maria Papini
The design of molecules mimicking biologically relevant glycans is a significant goal for understanding important biological processesand may lead to new therapeutic and diagnostic agents. We focused our attention on the trisaccharide human natural killer cell-1 (HNK-1), considered the antigenic determinant ofmyelin-associated glycoprotein and the target of clinically relevant auto-antibodies in autoimmune neurological disorders such as IgM monoclonal gammopathy and demyelinating polyneuropathy.We describe a structure-activity relationship study based on surface plasmon resonance binding affinities aiming at the optimization of a peptide mimicking the HNK-1 minimal epitope...
April 12, 2017: ChemMedChem
https://www.readbyqxmd.com/read/28397326/diagnosis-and-management-of-neuropathies-associated-with-plasma-cell-dyscrasias
#12
REVIEW
Evan Rosenbaum, Douglas Marks, Shahzad Raza
Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Fortunately, with increasing understanding into the biologic mechanisms of underlying hematologic diseases, additional biomarkers have recently been developed, thus improving our diagnostic capacity...
April 10, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28389623/neoplastic-plasma-cells-generate-an-inflammatory-environment-within-bone-marrow-and-markedly-alter-the-distribution-of-t-cells-between-lymphoid-compartments
#13
Oliver C Goodyear, Sarah Essex, Anandram Seetharam, Supratik Basu, Paul Moss, Guy Pratt
Monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM) are characterised by the accumulation of malignant plasma cells within bone marrow and lead to a range of abnormalities in the peripheral blood T cell repertoire. We investigated the level of inflammatory chemokines within the bone marrow and blood of patients with MGUS and MM and related this to the pattern of chemokine receptor expression on T cells in both compartments.The expression of a wide range of chemokine ligands for CXCR3 and CCR4 was markedly increased within the bone marrow of patients with MGUS and MM compared to healthy donors...
March 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28384387/a-first-czech-analysis-of-1887-cases-with-monoclonal-gammopathy-of-undetermined-significance
#14
Roman Hájek, Viera Sandecká, Zdeněk Adam, Ivan Špička, Vlastimil Ščudla, Evžen Gregora, Jakub Radocha, Lenka Walterová, Petr Kessler, Lenka Zahradová, Dagmar Adamová, Kamila Valentova, Ivan Vonke, Jarmila Obernauerová, David Starostka, Marek Wróbel, Lucie Brožová, Jiří Jarkovský, Aneta Mikulášová, Lucie Říhová, Sabina Ševčíková, Marta Krejčí, Ján Straub, Jiří Minařík, Luděk Pour, Zdeněk Král, Vladimír Maisnar
INTRODUCTION: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant condition with a risk of malignant conversion. PATIENTS AND METHODS: With the aim to estimate the cumulative risk MGUS progression into hematologic malignancies, we analyzed a nationwide population-based cohort of 1887 MGUS patients from the Czech Registry of Monoclonal Gammopathies (RMG) between 2007 and 2013. RESULTS: During the follow-up period (median 4 years; range 0...
April 6, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28381142/crystal-storing-histiocytosis
#15
Preithy Uthamalingam, Sangita Mehta
Crystal-storing histiocytosis (CSH) is a rare histiocytic lesion, most often described in association with lymphoid malignancies, especially plasma cell myeloma or lymphomas associated with monoclonal gammopathy. A few cases have also been described in patients without an underlying lymphoid/plasmacytic neoplasm. The histiocytes are characterized by intralysosomal accumulation of crystals composed of whole or part of the immunoglobulin molecule. The pathobiology is largely unclear. It is a rare phenomenon and the available literature is restricted to case reports and a few case series...
February 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28380655/-paraproteinemic-keratopathy-as-a-clinical-sign-of-monoclonal-gammopathy
#16
J Wasielica-Poslednik, A Gericke, N Pfeiffer, W Lisch
Patients with monoclonal gammopathy can show paraproteinemic keratopathy (PPK) with an indication to treatment. PPK has to be differentiated from corneal dystrophies, systemic metabolic disorders with corneal involvement, as well as from immunologic and inflammatory corneal diseases.
April 5, 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28375557/genome-wide-association-study-on-monoclonal-gammopathy-of-unknown-significance-mgus
#17
Hauke Thomsen, Chiara Campo, Niels Weinhold, Miguel Inacio da Silva Filho, Luděk Pour, Evžen Gregora, Pavel Vodicka, Ludmila Vodickova, Per Hoffmann, Markus M Nöthen, Karl-Heinz Jöckel, Christian Langer, Roman Hajek, Hartmut Goldschmidt, Kari Hemminki, Asta Försti
OBJECTIVES: To identify germline variants contributing to the development of monoclonal gammopathy of undetermined significance (MGUS), an asymptomatic pre-malignant precursor for multiple myeloma (MM). METHODS: We conducted the first genome-wide association study (GWAS) on MGUS on 243 German cases with a replication on 294 Czech cases. Identified loci were further analyzed in 1508 German MM patients. New MM loci recently reported in a meta-analysis were also tested in the MGUS GWAS...
April 4, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28371346/dramatic-resolution-of-disseminated-pyoderma-gangrenosum-associated-with-monoclonal-gammopathy-after-therapy-with-bortezomib-and-dexamethasone
#18
Virginia Velasco-Tamariz, Gonzalo Carreño-Tarragona, Fátima Tous-Romero, Elena Gil-de la Cruz, Estela Martín-Clavero, Raquel Rivera-Díaz
Pyoderma gangrenosum (PG) is an uncommon inflammatory and ulcerative skin disorder, which is commonly associated with systemic conditions such as inflammatory bowel disease, arthritis and haematological malignancies. It is widely stated that control of the underlying diseases may lead to resolution of PG. However, standard of care dictates that patients suffering with monoclonal gammopathy of undetermined significance or smouldering multiple myeloma (MM) should not receive therapy unless they progress to symptomatic MM...
March 29, 2017: International Wound Journal
https://www.readbyqxmd.com/read/28371144/laminar-crystal-deposition-in-large-vessels-in-a-patient-with-crystalglobulinemia
#19
Yukako Shintani-Domoto, Aya Shinozaki-Ushiku, Hidemi Okuma, Mineo Kurokawa, Masashi Fukayama
Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma...
March 31, 2017: Pathology International
https://www.readbyqxmd.com/read/28369766/method-for-dna-ploidy-analysis-along-with-immunophenotyping-for-rare-populations-in-a-sample-using-fxcycle-violet
#20
Prashant Tembhare, Yajamanam Badrinath, Sitaram Ghogale, Papagudi Ganesan Subramanian
The clinical use of flow cytometric DNA ploidy assay has been extended towards stratifying the risk of diseases, such as monoclonal gammopathies or B cell acute lymphoblastic leukemia, and to detect circulating tumor cells, both of which require detection of minute cell populations. This unit describes a protocol for determining DNA ploidy in fixed samples with simultaneous surface immunophenotyping. It is an easy method for simultaneous 6- to 8-color immunophenotyping and DNA content analysis using FxCycle Violet (FCV; DAPI) dye...
April 3, 2017: Current Protocols in Cytometry
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