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Monoclonal gammopathy of undetermined significance

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https://www.readbyqxmd.com/read/29183887/how-i-manage-monoclonal-gammopathy-of-undetermined-significance
#1
Ronald S Go, S Vincent Rajkumar
Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is intentional and credited to hematologists, the discovery of MGUS is most often incidental and made by non-hematologists. MGUS is considered an obligate precursor to several lymphoplasmacytic malignancies including immunoglobulin light chain amyloidosis, multiple myeloma, and Waldenström macroglobulinemia. Therefore, long-term follow-up is generally recommended...
November 28, 2017: Blood
https://www.readbyqxmd.com/read/29183861/bilateral-striopallidodentate-calcinosis-associated-with-sj%C3%A3-gren-s-syndrome-and-igd%C3%AE-monoclonal-gammopathy-of-undetermined-significance
#2
Taro Horino, Tatsuki Matsumoto, Kosuke Inoue, Osamu Ichii, Yoshio Terada
We presented the first case of bilateral striopallidodentate calcinosis secondary to Sjögren's syndrome. Further consideration should be given to the association between Sjögren's syndrome and bilateral striopallidodentate calcinosis, because Sjögren's syndrome is latent, but more frequent than other autoimmune diseases.
November 25, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29180919/false-positive-immunoassay-for-heparin-induced-thrombocytopenia-in-the-presence-of-monoclonal-gammopathy-a-case-report
#3
Ivana Markovic, Zeljko Debeljak, Bojana Bosnjak, Maja Marijanovic
Heparin induced thrombocytopenia (HIT) is a life-threatening disorder which diagnosis depends on laboratory evaluation. The objective of this report is to present the impact of different laboratory methods for HIT detection on the diagnostic evaluation process. In this case, a 78-year old female patient previously diagnosed with monoclonal gammopathy of undetermined significance (MGUS) was administered with heparin for pulmonary embolism treatment. Patient's initial diagnostic work-up (determination of platelet count and prothrombin time measurement for monitoring of pharmacotherapy) was followed by the clinical estimation of HIT likelihood by "4Ts" score, two immunoassays (ID-PaGIA Heparin/PF4 Antibody Test and ELISA PF4 IgG assay) and one functional test called high-performance liquid chromatography serotonin release assay (HPLC-SRA)...
October 15, 2017: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/29176541/diffuse-peritoneal-and-bowel-wall-infiltration-by-light-chain-al-amyloidosis-with-omental-calcification-mimicking-abdominal-carcinomatosis-an-elderly-female-with-incidental-finding-of-light-chain-monoclonal-gammopathy-of-undetermined-significance-lc-mgus
#4
Shoaib Junejo, Yasir Ali, Sandeep Singh Lubana, Sandeep S Tuli
BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS)...
November 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29153091/detection-of-complex-genomic-signatures-associated-with-risk-in-plasma-cell-disorders
#5
Nadine K Berry, Amanda Dixon-McIver, Rodney J Scott, Philip Rowlings, Anoop K Enjeti
Plasma cell disorders (PCD) range from benign to highly malignant disease. The ability to detect risk-stratifying aberrations based on cytogenetic and molecular genetic assays plays an increasing role in therapeutic decision making. In this study, 58 patients were chosen for screening by comparative genomic hybridisation microarray (aCGH) to identify the new high-risk prognostic markers of chromothripsis and chromoanasynthesis. All patients had an unequivocal clinical diagnosis of a plasma cell disorder (plasma cell myeloma (PCM)(n = 51) or monoclonal gammopathy of undetermined significance (MGUS)(n = 7)) and an abnormal FISH result...
December 2017: Cancer Genetics
https://www.readbyqxmd.com/read/29146225/response-comparison-of-multiple-myeloma-and-monoclonal-gammopathy-of-undetermined-significance-to-the-same-anti-myeloma-therapy-a-retrospective-cohort-study
#6
John P Campbell, Jennifer L J Heaney, Sankalp Pandya, Zaheer Afzal, Martin Kaiser, Roger Owen, J Anthony Child, David A Cairns, Walter Gregory, Gareth J Morgan, Graham H Jackson, Chris M Bunce, Mark T Drayson
BACKGROUND: Multiple myeloma is consistently preceded by monoclonal gammopathy of undetermined significance (MGUS), which is usually only treated by a form of anti-multiple myeloma therapy if it is causing substantial disease through deposition of secreted M proteins. However, studies comparing how MGUS and multiple myeloma plasma cell clones respond to these therapies are scarce. Biclonal gammopathy multiple myeloma is characterised by the coexistence of an active multiple myeloma clone and a benign MGUS clone, and thus provides a unique model to assess the responses of separate clones to the same anti-multiple myeloma therapy, in the same patient, at the same time...
November 13, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/29137341/clinicopathological-significance-of-the-p16-hypermethylation-in-multiple-myeloma-a-systematic-review-and-meta-analysis
#7
Huiqing Yu, Liejun Yang, Yunfeng Fu, Meng Gao, Ling Tian
It is well known that the loss of function of the p16INK4A gene is mainly caused by the hypermethylation of the p16 gene; however, whether or not the inactivation is associated with the clinical significance of multiple myeloma (MM) remains elusive. A meta-analysis was conducted to quantitatively determine the role of the p16 hypermethylation in the clinical significance of MM. We demonstrated that MM patients show much higher hypermethylation rates on the p16 gene in bone marrow compared to normal individuals, as well as monoclonal gammopathy of undetermined significance (MGUS)...
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29137262/disease-spectrum-of-abnormal-serum-free-light-chain-ratio-and-its-diagnostic-significance
#8
Bin Xu, Yi Tang, Jianfeng Zhou, Peiling Zhang, Huijun Li
Objective: To analyze the spectrum of abnormal serum free light chain ratio (sFLC κ/λ ratio), and to redefine the range of sFLC κ/λ ratio, so as to achieve hierarchical diagnosis of diseases with abnormal sFLC κ/λ ratio, resulting in the increased sensitivity and specificity in the diagnosis of monoclonal plasma diseases. Methods: Enrolled 1,340 patients with abnormal sFLC κ/λ ratio (<0.26 or >1.65) were grouped: (1) group A: malignant plasma diseases; (2) group B: monoclonal gammopathies of undetermined significance (MGUS); (3) group C: reactive plasma diseases...
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29112785/associated-conditions-in-small-fiber-neuropathy-a-large-cohort-study-and-review-of-the-literature
#9
Bianca T A de Greef, Janneke G J Hoeijmakers, Carla M L Gorissen-Brouwers, Margot Geerts, Catharina G Faber, Ingemar S J Merkies
BACKGROUND: Small fiber neuropathy (SFN) is a common disorder, leading to neuropathic pain and autonomic symptoms. The objective of this study was to investigate associated conditions in a large cohort of SFN patients and compare the prevalence to healthy individuals. METHODS: A total of 921 patients with pure SFN were screened according to a standardized comprehensive diagnostic algorithm and compared with literature findings. RESULTS: In 53% of the patients, no associated condition could be found...
November 7, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29098000/pro-inflammatory-state-in-monoclonal-gammopathy-of-undetermined-significance-and-in-multiple-myeloma-is-characterized-by-low-sialylation-of-pathogen-specific-and-other-monoclonal-immunoglobulins
#10
Adrien Bosseboeuf, Sophie Allain-Maillet, Nicolas Mennesson, Anne Tallet, Cédric Rossi, Laurent Garderet, Denis Caillot, Philippe Moreau, Eric Piver, François Girodon, Hélène Perreault, Sophie Brouard, Arnaud Nicot, Edith Bigot-Corbel, Sylvie Hermouet, Jean Harb
Multiple myeloma (MM) and its pre-cancerous stage monoclonal gammopathy of undetermined significance (MGUS) allow to study immune responses and the chronology of inflammation in the context of blood malignancies. Both diseases are characterized by the production of a monoclonal immunoglobulin (mc Ig) which for subsets of MGUS and MM patients targets pathogens known to cause latent infection, a major cause of inflammation. Inflammation may influence the structure of both polyclonal (pc) Ig and mc Ig produced by malignant plasma cells via the sialylation of Ig Fc fragment...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29079358/monoclonal-protein-reference-change-value-as-determined-by-gel-based-serum-protein-electrophoresis
#11
Mina Salamatmanesh, Christopher R McCudden, Arleigh McCurdy, Ronald A Booth
INTRODUCTION: The International Myeloma Working Group recommendations for monitoring disease progression or response include quantitation of the involved monoclonal immunoglobulin. They have defined the minimum change criteria of ≧25% with an absolute change of no <5g/L for either minimal response or progression. Limited evidence is available to accurately determine the magnitude of change in a monoclonal protein to reflect a true change in clinical status. Here we determined the analytical and biological variability of monoclonal proteins in stable monoclonal gammopathy of undetermined significance (MGUS) patients...
October 24, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/29070644/anti-mag-antibodies-in-202-patients-clinicopathological-and-therapeutic-features
#12
Juliette Svahn, Philippe Petiot, Jean-Christophe Antoine, Christophe Vial, Emilien Delmont, Karine Viala, Andreas J Steck, Armelle Magot, Cecile Cauquil, Aline Zarea, Andoni Echaniz-Laguna, Ruxandra Iancu Ferfoglia, Antoine Gueguen, Laurent Magy, Jean-Marc Léger, Thierry Kuntzer, Karine Ferraud, Arnaud Lacour, Jean-Philippe Camdessanché
OBJECTIVE: To assess the clinicopathological and therapeutic features of patients with low (≥1000 to <10 000 Bühlmann Titre Units) (BTU), medium (10 000-70 000) or high (≥70 000) anti-myelin-associated glycoprotein (anti-MAG) antibody titres. METHODS: We retrospectively and prospectively analysed standardised report forms and medical records of 202 patients from 14 neuromuscular centres. RESULTS: Mean age at onset and mean time between symptom onset to last follow-up were respectively 62...
October 25, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29053158/prevalence-of-myeloma-precursor-state-monoclonal-gammopathy-of-undetermined-significance-in-12372-individuals-10-49-years-old-a-population-based-study-from-the-national-health-and-nutrition-examination-survey
#13
O Landgren, B I Graubard, S Kumar, R A Kyle, J A Katzmann, K Murata, R Costello, A Dispenzieri, N Caporaso, S Mailankody, N Korde, M Hultcrantz, T M Therneau, D R Larson, J R Cerhan, S V Rajkumar
We studied the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in younger individuals, age 10-49 years, using samples from the National Health and Nutritional Examination Survey (NHANES) III. NHANES prevalence rates were standardized to the 2000 US total population. Among 12 372 individuals (4073 blacks, 4146 Mexican-Americans, 3595 whites, and 558 others), MGUS was identified in 63 persons (0.34%, 95% CI 0.23-0.50). The prevalence of MGUS was significantly higher in blacks (0.88%, 95% CI 0...
October 20, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29036799/hemorheological-parameters-in-monoclonal-gammopathy-of-undetermined-significance-mgus
#14
G Caimi, E Hopps, M Carlisi, M Montana, E Gallà, R Lo Presti, S Siragusa
There is scarcity of information about the hemorheological pattern in subjects with Monoclonal Gammopathy of Undetermined Significance (MGUS). This preliminary research is focused on the behaviour of whole-blood and plasma viscosity, haematocrit and erythrocyte deformability in the above clinical condition. We enrolled 21 MGUS subjects (10 women and 11 men; mean age 66.4 ± 11.6 years). In fasting venous blood we examined whole-blood and plasma viscosity at high and low shear rates, haematocrit, the ratios between whole-blood viscosity (at high and low shear rate) and haematocrit × 100, the ratio between plasma viscosity at low and high shear rate, and the erythrocyte deformability expressed as elongation index...
October 7, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#15
REVIEW
Eduardo Nobile-Orazio, Mariangela Bianco, Andrea Nozza
Purpose of review Several advances have been made on the pathogenesis and therapy of neuropathies associated with paraproteinemia (monoclonal gammopathy). It is important for the neurologist to understand the pathogenetic relevance of this association especially when the hematological disease does not require per se any therapy. Recent findings Treatment of the neuropathy in patients with malignant paraproteinemia is mainly addressed by the hematologist while the neurologist is mainly involved in the initial diagnosis and in deciding whether the neuropathy is caused by the disease or by the chemotherapy used for the disease...
October 16, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/29031125/low-protein-z-levels-in-patients-with-plasma-cell-neoplasms-are-inversely-correlated-with-il-6-levels
#16
O Gutwein, N Rahimi-Levene, K Herzog-Tzarfati, O Garach-Jehoshua, A Nagler, M Izak, M Koren-Michowitz
Patients with multiple myeloma (MM) have an increased thrombotic risk, but pathogenesis remains uncertain. Low levels of Protein Z (PZ), a vitamin K-dependent plasma protein, are associated with venous as well as arterial thrombosis. The purpose of this study was to analyze PZ levels in patients with plasma cell neoplasms. PATIENTS AND METHODS: The study consisted of 64 plasma cells neoplasm patients and 42 healthy individuals. Clinical investigations included measurement of plasma PZ and IL-6 levels...
November 2017: Leukemia Research
https://www.readbyqxmd.com/read/29027517/-unexpected-symptoms-of-monoclonal-gammopathy
#17
P M Smit, A C Abrahams, R G E Schutgens, N C Notermans, M C Minnema
Monoclonal gammopathy of undetermined significance (MGUS) is a common haematological disorder characterized by the presence of a monoclonal protein (M-protein). MGUS is considered an asymptomatic 'innocent' pre-malignant precursor condition of - mostly - multiple myeloma, without indication for treatment. We present three cases illustrating that MGUS can lead to serious problems. The first patient, a 51-year-old female, presented with polyneuropathy due to anti-MAG antibodies related to IgM MGUS. The second patient, a 37-year-old female, presented with proteinuria due to immunotactoid glomerulopathy caused by renal monoclonal IgG deposition associated with MGUS...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28983055/pattern-of-somatic-mutations-in-patients-with-waldenstr%C3%A3-m-macroglobulinemia-or-igm-monoclonal-gammopathy-of-undetermined-significance
#18
Marzia Varettoni, Silvia Zibellini, Irene Defrancesco, Virginia Valeria Ferretti, Ettore Rizzo, Luca Malcovati, Anna Gallì, Matteo Giovanni Della Porta, Emanuela Boveri, Luca Arcaini, Chiara Candido, Marco Paulli, Mario Cazzola
We analyzed MYD88 and CXCR4 mutation status of 260 patients with Waldenström macroglobulinemia or IgM monoclonal gammopathy of undetermined significance using allele-specific real time quantitative PCR and Sanger sequencing respectively. A subgroup of 119 patients was further studied with next-generation sequencing of 11 target genes (MYD88, CXCR4, ARID1-A, KMT2D, NOTCH2, TP53, PRDM1, CD79B, TRAF3, MYBBP1A, TNFAIP3). MYD88 (L265P) was found at diagnosis in 91% of patients with Waldenström macroglobulinemia and in 60% of patients with IgM monoclonal gammopathy of undetermined significance using allele-specific PCR...
October 5, 2017: Haematologica
https://www.readbyqxmd.com/read/28978808/monoclonal-igg-in-mgus-and-multiple-myeloma-targets-infectious-pathogens
#19
Adrien Bosseboeuf, Delphine Feron, Anne Tallet, Cédric Rossi, Cathy Charlier, Laurent Garderet, Denis Caillot, Philippe Moreau, Marina Cardó-Vila, Renata Pasqualini, Wadih Arap, Alfreda Destea Nelson, Bridget S Wilson, Hélène Perreault, Eric Piver, Pierre Weigel, François Girodon, Jean Harb, Edith Bigot-Corbel, Sylvie Hermouet
Subsets of mature B cell neoplasms are linked to infection with intracellular pathogens such as Epstein-Barr virus (EBV), hepatitis C virus (HCV), or Helicobacter pylori. However, the association between infection and the immunoglobulin-secreting (Ig-secreting) B proliferative disorders remains largely unresolved. We investigated whether the monoclonal IgG (mc IgG) produced by patients diagnosed with monoclonal gammopathy of undetermined significance (MGUS) or multiple myeloma (MM) targets infectious pathogens...
October 5, 2017: JCI Insight
https://www.readbyqxmd.com/read/28969692/role-of-protein-kinases-ck1%C3%AE-and-ck2-in-multiple-myeloma-regulation-of-pivotal-survival-and-stress-managing-pathways
#20
REVIEW
Sabrina Manni, Marilena Carrino, Francesco Piazza
Multiple myeloma (MM) is a malignant tumor of transformed plasma cells. MM pathogenesis is a multistep process. This cancer can occur de novo (rarely) or it can develop from monoclonal gammopathy of undetermined significance (most of the cases). MM can be asymptomatic (smoldering myeloma) or clinically active. Malignant plasma cells exploit intrinsic and extrinsic bone marrow microenvironment-derived growth signals. Upregulation of stress-coping pathways is also instrumental to maintain MM cell growth. The phylogenetically related Ser/Thr kinases CSNK1A1 (CK1α) and CSNK2 (CK2) have recently gained a growing importance in hematologic malignancies arising both from precursors and from mature blood cells...
October 2, 2017: Journal of Hematology & Oncology
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