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Monoclonal gammopathy of undetermined significance

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https://www.readbyqxmd.com/read/29450453/the-association-of-age-with-clinical-presentation-and-comorbidities-of-pyoderma-gangrenosum
#1
Hovik J Ashchyan, Daniel C Butler, Caroline A Nelson, Megan H Noe, William G Tsiaras, Stephen J Lockwood, William D James, Robert G Micheletti, Misha Rosenbach, Arash Mostaghimi
Importance: Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis. Current knowledge of this rare disease is limited owing to a lack of validated diagnostic criteria and large population studies. Objective: To evaluate the association of age with the clinical presentation and comorbidities of pyoderma gangrenosum. Design, Setting, and Participants: This was a multicenter retrospective cohort study performed at tertiary academic referral centers in urban settings...
February 14, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29448055/proteomic-characterization-of-withaferin-a-targeted-protein-networks-for-the-treatment-of-monoclonal-myeloma-gammopathies
#2
Martin Dom, Fritz Offner, Wim Vanden Berghe, Xaveer Van Ostade
Withaferin A (WA), a natural steroid lactone from the plant Withania somnifera, is often studied because of its antitumor properties. Although many in vitro and in vivo studies have been performed, the identification of Withaferin A protein targets and its mechanism of antitumor action remain incomplete. We used quantitative chemoproteomics and differential protein expression analysis to characterize the WA antitumor effects on a multiple myeloma cell model. Identified relevant targets were further validated by Ingenuity Pathway Analysis and Western blot and indicate that WA targets protein networks that are specific for monoclonal gammopathy of undetermined significance (MGUS) and other closely related disorders, such as multiple myeloma (MM) and Waldenström macroglobulinemia (WM)...
February 12, 2018: Journal of Proteomics
https://www.readbyqxmd.com/read/29424556/monoclonal-gammopathy-of-undetermined-significance-in-systemic-transthyretin-amyloidosis-attr
#3
Pooja Phull, Vaishali Sanchorawala, Lawreen H Connors, Gheorghe Doros, Frederick L Ruberg, John L Berk, Shayna Sarosiek
OBJECTIVE: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. PATIENTS AND METHODS: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016...
February 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29416776/monoclonal-gammopathy-of-renal-significance-mgrs-increases-the-risk-for-progression-to-multiple-myeloma-an-observational-study-of-2935-mgus-patients
#4
Normann Steiner, Georg Göbel, Patricia Suchecki, Wolfgang Prokop, Hannes Neuwirt, Eberhard Gunsilius
Purpose: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignancy preceding multiple myeloma (MM) or related disorders. In MGUS, renal impairment caused by deposition of the monoclonal immunoglobulins or free light-chains monoclonal gammopathy of renal significance (MGRS) is often associated with high morbidity and mortality. We analysed the prevalence of renal impairment, clinical features and the long-term outcome in 2935 patients with MGUS. Methods: Between 1/2000 and 8/2016, 2935 adult patients with MGUS were identified in our database...
January 5, 2018: Oncotarget
https://www.readbyqxmd.com/read/29390260/paraproteinemic-keratopathy-in-monoclonal-gammopathy-of-undetermined-significance-treated-with-primary-keratoprosthesis-case-report-histopathologic-findings-and-world-literature-review
#5
Homer H Chiang, Rebekah S Wieland, Thomas S Rogers, Pamela C Gibson, George Atweh, Gregory McCormick
RATIONALE: We report a case of paraproteinemic keratopathy associated with monoclonal gammopathy of undetermined significance, treated with keratoprosthesis as a primary penetrating procedure. Histopathological findings and a world literature review are presented. PATIENT CONCERNS: A 74 year old female recently diagnosed with monoclonal gammopathy undetermined significance presented with progressive blurry vision bilaterally. DIAGNOSES: Examination revealed corneal opacities consistent with paraproteinemic keratopathy...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29389935/novel-pedigree-analysis-implicates-dna-repair-and-chromatin-remodeling-in-multiple-myeloma-risk
#6
Rosalie G Waller, Todd M Darlington, Xiaomu Wei, Michael J Madsen, Alun Thomas, Karen Curtin, Hilary Coon, Venkatesh Rajamanickam, Justin Musinsky, David Jayabalan, Djordje Atanackovic, S Vincent Rajkumar, Shaji Kumar, Susan Slager, Mridu Middha, Perrine Galia, Delphine Demangel, Mohamed Salama, Vijai Joseph, James McKay, Kenneth Offit, Robert J Klein, Steven M Lipkin, Charles Dumontet, Celine M Vachon, Nicola J Camp
The high-risk pedigree (HRP) design is an established strategy to discover rare, highly-penetrant, Mendelian-like causal variants. Its success, however, in complex traits has been modest, largely due to challenges of genetic heterogeneity and complex inheritance models. We describe a HRP strategy that addresses intra-familial heterogeneity, and identifies inherited segments important for mapping regulatory risk. We apply this new Shared Genomic Segment (SGS) method in 11 extended, Utah, multiple myeloma (MM) HRPs, and subsequent exome sequencing in SGS regions of interest in 1063 MM / MGUS (monoclonal gammopathy of undetermined significance-a precursor to MM) cases and 964 controls from a jointly-called collaborative resource, including cases from the initial 11 HRPs...
February 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29384915/longitudinal-extensive-transverse-myelitis-with-an-abnormal-uflc-ratio-in-a-pediatric-patient-case-report-and-literature-review
#7
REVIEW
Po-Chang Hsu, Shyi-Jou Chen
RATIONALE: The serum and urine-free light chain (sFLC/uFLC) ratios of kappa (κ) to lambda (λ) serve as biomarkers for plasma cell disorders, especially multiple myeloma. However, to our best knowledge, the ratios have not been appropriately assessed for acute transverse myelitis (ATM). PATIENT CONCERNS: We present a 12-year-old boy who had sudden onset low back pain following paralysis of his 4 extremities and disturbance consciousness. Magnetic resonance imaging (MRI) of the brain and spine indicated diffuse hyperintensity in T2-weighted images from the cervical spinal cord to the conus medullaris...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29372735/isolation-of-circulating-plasma-cells-from-blood-of-patients-diagnosed-with-clonal-plasma-cell-disorders-using-cell-selection-microfluidics
#8
Joyce W Kamande, Maria A M Lindell, Małgorzata A Witek, Peter M Voorhees, Steven A Soper
Blood samples from patients with plasma cell disorders were analysed for the presence of circulating plasma cells (CPCs) using a microfluidic device modified with monoclonal anti-CD138 antibodies. CPCs were immuno-phenotyped using a CD38/CD56/CD45 panel and identified in 78% of patients with monoclonal gammopathy of undetermined significance (MGUS), all patients with smouldering and symptomatic multiple myeloma (MM), and none in the controls. The burden of CPCs was higher in patients with symptomatic MM compared with MGUS and smouldering MM (p < 0...
January 26, 2018: Integrative Biology: Quantitative Biosciences From Nano to Macro
https://www.readbyqxmd.com/read/29353304/unraveling-the-heterogeneity-of-igm-monoclonal-gammopathies-a-gene-mutational-and-gene-expression-study
#9
Cristina Jiménez, María Isabel Prieto-Conde, María García-Álvarez, Miguel Alcoceba, Fernando Escalante, María Del Carmen Chillón, Alfonso García de Coca, Ana Balanzategui, Alberto Cantalapiedra, Carlos Aguilar, Rocío Corral, Tomás González-López, Luis A Marín, Abelardo Bárez, Noemí Puig, Aránzazu García-Mateo, Norma C Gutiérrez, María Eugenia Sarasquete, Marcos González, Ramón García-Sanz
Immunoglobulin M (IgM) monoclonal gammopathies show considerable variability, involving three different stages of presentation: IgM monoclonal gammopathy of undetermined significance (IgM-MGUS), asymptomatic Waldenström's macroglobulinemia (AWM), and symptomatic WM (SWM). Despite recent findings about the genomic and transcriptomic characteristics of such disorders, we know little about the causes of this clinical heterogeneity or the mechanisms involved in the progression from indolent to symptomatic forms...
January 20, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29345175/a-role-for-bone-turnover-markers-%C3%AE-crosslaps-ctx-and-amino-terminal-propeptide-of-type-i-collagen-pinp-as-potential-indicators-for-disease-progression-from-mgus-to-multiple-myeloma
#10
Sonia Vallet, Nicholas R Hoyle, Robert A Kyle, Klaus Podar, Martin Pecherstorfer
Multiple myeloma (MM) is characterized by bone lesions arising due to unbalanced bone remodeling. Changes in the bone formation marker amino-terminal propeptide of type I collagen (PINP) and the bone resorption marker β-CrossLaps (CTX) reflect physiologic bone turnover. Whether PINP and CTX have a role in disease progression from monoclonal gammopathy of undetermined significance (MGUS) to MM is unknown. In this cross-sectional follow-up study, 241 patients with MM or MGUS were included. Serum levels of PINP and CTX were significantly higher in MM patients compared to MGUS...
January 18, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29342381/long-term-follow-up-of-monoclonal-gammopathy-of-undetermined-significance
#11
Robert A Kyle, Dirk R Larson, Terry M Therneau, Angela Dispenzieri, Shaji Kumar, James R Cerhan, S Vincent Rajkumar
BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) occurs in approximately 3% of persons 50 years of age or older. METHODS: We studied 1384 patients who were residing in southeastern Minnesota and in whom MGUS was diagnosed at the Mayo Clinic in the period from 1960 through 1994; the median follow-up was 34.1 years (range, 0.0 to 43.6). The primary end point was progression to multiple myeloma or another plasma-cell or lymphoid disorder. RESULTS: During 14,130 person-years of follow-up, MGUS progressed in 147 patients (11%), a rate that was 6...
January 18, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29330758/-monoclonal-gammopathy-of-undetermined-significance-and-multiple-myeloma
#12
REVIEW
M Schmalzing, H-P Tony, S Knop
In rheumatological practice monoclonal gammopathy of undetermined significance (MGUS) is a common incidental finding. Several rheumatic inflammatory diseases are known to have an elevated risk of MGUS, which can evolve into multiple myeloma or other lymphatic malignancies. The relevant definitions of disease entities are described, as well as algorithms for further diagnostic work-up and follow-up for monoclonal gammopathy, depending on the risk of progression. Therapeutic strategies against multiple myeloma are presented...
October 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/29326807/heavy-chain-diseases-and-myeloma-associated-fanconi-syndrome-an-update
#13
REVIEW
Roberto Ria, Franco Dammacco, Angelo Vacca
The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the production of a monoclonal immunoglobulin heavy chain without an associated light chain. There are three types of HCD, defined by the class of immunoglobulin heavy chain produced: IgA (α-HCD), IgG (γ-HCD), and IgM (μ-HCD). Alpha-HCD is the most common and usually occurs as intestinal malabsorption in a young adult from a country of the Mediterranean area. Gamma- and μ-HCDs are rarer and associated with a B-cell non-Hodgkin lymphoma that produces an abnormal Ig heavy chain...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29323095/intracytoplasmic-inclusions-in-plasma-cells-a-diagnostic-adjunct-in-monoclonal-gammopathy-of-undetermined-significance
#14
Smeeta Gajendra, Rashi Sharma, Swachi Jain, Ritesh Sachdev, Nitin Sood
No abstract text is available yet for this article.
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29316245/flow-cytometric-aberrancies-in-plasma-cell-myeloma-and-mgus-correlation-with-laboratory-parameters
#15
Sarika Gupta, Nitin J Karandikar, Timothy Ginader, Andrew M Bellizzi, Carol J Holman
BACKGROUND: Multiparametric flow cytometry (MFC) is a useful tool for diagnosis of plasma cell dyscrasias and assessment of minimal residual disease (MRD) in plasma cell myeloma (PCM). However, the immunophenotypic differences between the clonal plasma cells (PCs) of plasma cell myeloma (PCM) and those of monoclonal gammopathy of undetermined significance (MGUS) as well as the correlation of these flow cytometric markers with pertinent laboratory parameters have not been evaluated. METHODS: We retrospectively identified all newly diagnosed treatment-naive PCM and MGUS patients between 09/2014 and 06/2015 who underwent 10-color flow-cytometric evaluation: CD45, CD38, CD138, cKappa, cLambda, CD19, CD27, CD28, CD56, CD117...
January 6, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29311715/the-bone-marrow-niche-in-mds-and-mgus-implications-for-aml-and-mm
#16
REVIEW
Irene M Ghobrial, Alexandre Detappe, Kenneth C Anderson, David P Steensma
Several haematological malignancies, including multiple myeloma (MM) and acute myeloid leukaemia (AML), have well-defined precursor states that precede the development of overt cancer. MM is almost always preceded by monoclonal gammopathy of undetermined significance (MGUS), and at least a quarter of all patients with myelodysplastic syndromes (MDS) have disease that evolves into AML. In turn, MDS are frequently anteceded by clonal haematopoiesis of indeterminate potential (CHIP). The acquisition of additional genetic and epigenetic alterations over time clearly influences the increasingly unstable and aggressive behaviour of neoplastic haematopoietic clones; however, perturbations in the bone-marrow microenvironment are increasingly recognized to have key roles in initiating and supporting oncogenesis...
January 9, 2018: Nature Reviews. Clinical Oncology
https://www.readbyqxmd.com/read/29296931/bone-disease-in-monoclonal-gammopathy-of-undetermined-significance-results-from-a-screened-population-based-study
#17
Sigrun Thorsteinsdottir, Sigrun H Lund, Ebba K Lindqvist, Marianna Thordardottir, Gunnar Sigurdsson, Rene Costello, Debra Burton, Hlif Steingrimsdottir, Vilmundur Gudnason, Gudny Eiriksdottir, Kristin Siggeirsdottir, Tamara B Harris, Ola Landgren, Sigurdur Y Kristinsson
Previous studies have shown that individuals with monoclonal gammopathy of undetermined significance (MGUS) have an increased risk of fractures, although the underlying mechanisms remain unknown. Our aim was to analyze bone mineral density (BMD), bone volume, and risk of fractures among individuals with MGUS. We performed a screening using the Age, Gene/Environment Susceptibility (AGES)-Reykjavik Study cohort, consisting of 5764 elderly individuals, identifying 300 individuals with MGUS, and 275 with light-chain MGUS...
December 26, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296866/obesity-and-risk-of-monoclonal-gammopathy-of-undetermined-significance-and-progression-to-multiple-myeloma-a-population-based-study
#18
Marianna Thordardottir, Ebba K Lindqvist, Sigrun H Lund, Rene Costello, Debra Burton, Neha Korde, Sham Mailankody, Gudny Eiriksdottir, Lenore J Launer, Vilmundur Gudnason, Tamara B Harris, Ola Landgren, Sigurdur Y Kristinsson
All multiple myeloma (MM) cases are preceded by the premalignant state monoclonal gammopathy of undetermined significance (MGUS). Results from previous studies show a positive association between obesity and MM; however, the association between obesity and MGUS is controversial. The aims were to determine (1) if obesity is associated with an increased risk of MGUS and light-chain MGUS (LC-MGUS) and (2) whether obesity is associated with a higher risk of progression to MM and other lymphoproliferative (LP) diseases...
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29281132/monoclonal-gammopathies-electronic-subspecialty-consultation
#19
Nicholas Burwick, Jacob Stein, David A Garcia, Virginia C Broudy, Robert E Richard
IMPORTANCE: Electronic consultation (e-consult) is an important component of care for patients in the Veterans Health Administration who require sub-specialty consultation but not urgent face to face evaluation. Monoclonal gammopathy of undetermined significance (MGUS) is a common reason for e-consult. While often benign, MGUS requires careful evaluation and persistent surveillance over time. OBJECTIVE: To identify areas to improve MGUS care delivery by e-consult...
December 27, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29250691/nephrotic-syndrome-secondary-to-amyloidosis-in-a-patient-with-monoclonal-gammopathy-with-renal-significance-mgrs
#20
Andrei Niculae, Ileana Peride, Vlad Vinereanu, Daniela Rădulescu, Ovidiu Gabriel Bratu, Bogdan Florin Geavlete, Ionel Alexandru ChecheriŢă
Monoclonal gammopathy with renal significance (MGRS) is a relative new-described entity, diagnosed especially in older patients and deriving from the group with monoclonal gammopathy of undetermined significance (MGUS). Various renal lesions may arise in MGRS, according to the ultrastructural characteristics of the monoclonal immunoglobulin deposition in the kidney, from proliferative glomerulopathies and amyloidosis to light chain proximal tubulopathy and crystal-storing histiocytosis. Although both are considered premalign or non-malignant hematological conditions, kidney involvement in MGRS aggravates the prognosis of the patients and need to be treated aggressively...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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