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Light chain amyloidosis

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https://www.readbyqxmd.com/read/28434927/impact-of-autologous-stem-cell-transplantation-on-the-incidence-and-outcome-of-oligoclonal-bands-in-patients-with-light-chain-amyloidosis
#1
Luis Gerardo Rodríguez-Lobato, Carlos Fernández de Larrea, M Teresa Cibeira, Natalia Tovar, Juan I Aróstegui, Laura Rosiñol, Tania Díaz, Ester Lozano, Montserrat Elena, Jordi Yagüe, Joan Bladé
The emergence of oligoclonal bands (OB) in patients with multiple myeloma achieving a complete remission (CR) after autologous stem cell transplantation (ASCT) and the use of novel agents is a well-recognized event. The presence of OB is associated with favorable outcome. However, the emergence of OB in light-chain (AL) amyloidosis has never been investigated. The aim of the study was to determine the incidence, natural history and prognostic impact of OB in 50 patients with AL amyloidosis who achieved at least a partial response (PR) either after up-front ASCT (20 patients [40%]) or after conventional treatment in patients ineligible for transplant (30 patients [60%])...
April 18, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28434368/outcome-of-very-young-%C3%A2-40-years-patients-with-immunoglobulin-light-chain-al-amyloidosis
#2
Jithma P Abeykoon, Jonas Paludo, Angela Dispenzieri, Morie A Gertz, David Dingli, Francis K Baudi, Wilson I Gonsalves, Robert A Kyle, Martha Q Lacy, Suzanne R Hayman, Nelson Leung, Taxiarchis Kourelis, S Vincent Rajkumar, Shaji Kumar, Prashant Kapoor
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434343/patients-with-al-amyloidosis-and-low-free-light-chain-burden-have-distinct-clinical-features-and-outcome
#3
Paolo Milani, Marco Basset, Francesca Russo, Andrea Foli, Francesca Lavatelli, Mario Nuvolone, Giovanni Ferraro, Giovanni Palladini, Giampaolo Merlini
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434321/renal-outcomes-of-autologous-stem-cell-transplantation-among-patients-with-light-chain-amyloidosis-a-single-centre-spanish-experience
#4
Sebastián Ernesto Azorín, María Teresa Cibeira, Manel Solé, Carlos Fernández de Larrea, Laura Rosiñol, Montse Rovira, Josep Maria Campistol, Joan Bladé
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434310/diagnostic-accuracy-of-new-indirect-elisas-for-free-light-chain-quantification-in-fat-tissue-of-patients-with-al-amyloidosis
#5
Elisabeth Eelsing, Johan Bijzet, Bouke Hazenberg
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434299/first-report-of-myd88-l265p-somatic-mutation-in-igm-associated-light-chain-amyloidosis
#6
Rajshekar Chakraborty, Anne J Novak, Stepen M Ansell, Eli Muchtar, Prashant Kapoor, Suzanne R Hayman, Angela Dispenzieri, Francis K Buadi, Martha Q Lacy, Rebecca L King, Morie A Gertz
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434293/severity-and-reversibility-of-cardiac-dysfunction-and-residual-concentration-of-amyloidogenic-light-chain-predict-overall-survival-of-patients-with-al-amyloidosis-who-attain-complete-response
#7
Giovanni Palladini, Paolo Milani, Marco Basset, Francesca Russo, Francesca Lavatelli, Mario Nuvolone, Giovanni Ferraro, Margherita Bozzola, Andrea Foli, Stefano Perlini, Giampaolo Merlini
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#8
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28394369/revisiting-conditioning-dose-in-newly-diagnosed-light-chain-amyloidosis-undergoing-frontline-autologous-stem-cell-transplant-impact-on-response-and-survival
#9
N Tandon, E Muchtar, S Sidana, A Dispenzieri, M Q Lacy, D Dingli, F K Buadi, S R Hayman, R Chakraborty, W J Hogan, W Gonsalves, R Warsame, T V Kourelis, N Leung, P Kapoor, S K Kumar, M A Gertz
Autologous stem cell transplantation (ASCT) is an important treatment modality in light chain (AL) amyloidosis. Use of reduced-dose melphalan conditioning is common, given the associated organ and functional decline. The impact of full-intensity melphalan conditioning (n=314) was compared to reduced-dose conditioning (n=143). Patients in the full-intensity group were younger, with better performance status, fewer involved organs, lower tumor burden and lower Mayo stage. Full-dose conditioning was associated with higher rate of very good partial response or better (79% vs 62%; P<0...
April 10, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28384285/impact-of-genotype-and-phenotype-on-cardiac-biomarkers-in-patients-with-transthyretin-amyloidosis-report-from-the-transthyretin-amyloidosis-outcome-survey-thaos
#10
Arnt V Kristen, Mathew S Maurer, Claudio Rapezzi, Rajiv Mundayat, Ole B Suhr, Thibaud Damy
AIM: Cardiac troponins and natriuretic peptides are established for risk stratification in light-chain amyloidosis. Data on cardiac biomarkers in transthyretin amyloidosis (ATTR) are lacking. METHODS AND RESULTS: Patients (n = 1617) with any of the following cardiac biomarkers, BNP (n = 1079), NT-proBNP (n = 550), troponin T (n = 274), and troponin I (n = 108), available at baseline in the Transthyretin Amyloidosis Outcomes Survey (THAOS) were analyzed for differences between genotypes and phenotypes and their association with survival...
2017: PloS One
https://www.readbyqxmd.com/read/28381753/the-coexistence-of-multiple-myeloma-associated-amyloid-light-chain-amyloidosis-and-fabry-disease-in-a-hemodialysis-patient
#11
Kensei Taguchi, Atsuo Moriyama, Goh Kodama, Yosuke Nakayama, Kei Fukami
Fabry disease (FD) is an inherited lysosomal disorder caused by an X-linked α-galactosidase A deficiency. We report the case of a 50-year-old male FD patient on hemodialysis who presented with macroglossia-related speaking difficulty and gastrointestinal symptoms. An endoscopic analysis revealed multiple gastric ulcers, and a histological examination led to a diagnosis of amyloid light-chain amyloidosis. Serum free light-chain and bone marrow analyses detected multiple myeloma (MM). Treatment with bortezomib and dexamethasone significantly improved the patient's symptoms...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28373528/addressing-common-questions-encountered-in-the-diagnosis-and-management-of-cardiac-amyloidosis
#12
REVIEW
Mathew S Maurer, Perry Elliott, Raymond Comenzo, Marc Semigran, Claudio Rapezzi
Advances in cardiac imaging have resulted in greater recognition of cardiac amyloidosis in everyday clinical practice, but the diagnosis continues to be made in patients with late-stage disease, suggesting that more needs to be done to improve awareness of its clinical manifestations and the potential of therapeutic intervention to improve prognosis. Light chain cardiac amyloidosis, in particular, if recognized early and treated with targeted plasma cell therapy, can be managed very effectively. For patients with transthyretin amyloidosis, there are numerous therapies that are currently in late-phase clinical trials...
April 4, 2017: Circulation
https://www.readbyqxmd.com/read/28367947/a-case-of-amyloid-myopathy-diagnosed-during-the-treatment-of-myopathy-associated-with-anti-signal-recognition-particle-antibodies
#13
Nobuko Kawakami, Yusuke Katsuyama, Yuka Hagiwara, Hidefumi Yoshida, Kang Kim, Kiyoshi Harada
A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Concomitant therapy with prednisolone and azathioprine caused the serum CK level to return to normal and it caused clinical manifestations to abate. One year later, however, muscle weakness worsened...
March 30, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28360189/biobanking-strategy-and-sample-preprocessing-for-integrative-research-in-monoclonal-gammopathies
#14
T Ševčíková, K Growková, Z Kufová, J Filipová, P Vrublová, T Jelínek, Z Kořístek, F Kryukov, E Kryukova, R Hájek
AIMS: Some types of monoclonal gammopathies are typified by a very limited availability of aberrant cells. Modern research use high throughput technologies and an integrated approach for detailed characterisation of abnormal cells. This strategy requires relatively high amounts of starting material which cannot be obtained from every diagnosis without causing inconvenience to the patient. The aim of this methodological paper is to reflect our long experience with laboratory work and describe the best protocols for sample collection, sorting and further preprocessing in terms of the available number of cells and intended downstream application in monoclonal gammopathies research...
March 30, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28355725/-a-case-report-of-immunoglonulin-%C3%AE-light-chain-amyloidosis-combined-with-leukocyte-cell-derived-chemotaxin-2-amyloidosis
#15
N An, S L Chen, X Li
No abstract text is available yet for this article.
April 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#16
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28350808/differential-recruitment-efficacy-of-patient-derived-amyloidogenic-and-myeloma-light-chain-proteins-by-synthetic-fibrils-a-metric-for-predicting-amyloid-propensity
#17
Emily B Martin, Angela Williams, Craig Wooliver, R Eric Heidel, Sarah Adams, John Dunlap, Marina Ramirez-Alvarado, Luis M Blancas-Mejia, Ronald H Lands, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Monoclonal free light chain (LC) proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL) amyloidosis and multiple myeloma (MM). Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular matrix proteins found in all patients with AL and in ~10-30% of patients who presented with MM. Amyloid deposits systemically in multiple organs and tissues leading to dysfunction and ultimately death...
2017: PloS One
https://www.readbyqxmd.com/read/28343171/circulating-free-light-chain-measurement-in-the-diagnosis-prognostic-assessment-and-evaluation-of-response-of-al-amyloidosis-comparison-of-freelite-and-n-latex-flc-assays
#18
Giovanni Palladini, Arnaud Jaccard, Paolo Milani, David Lavergne, Andrea Foli, Sebastien Bender, Francesca Lavatelli, Tiziana Bosoni, Veronica Valentini, Laura Pirolini, Giovanni Ferraro, Marco Basset, Francesca Russo, Mario Nuvolone, Riccardo Albertini, Michel Cogne, Giampaolo Merlini
BACKGROUND: The measurement of circulating free light chain (FLC) is essential in the diagnosis, prognostic stratification and evaluation of response to therapy in light chain (AL) amyloidosis. For more than 10 years, this has been done with an immunonephelometric assay based on polyclonal antibodies (Freelite), and cutoffs for staging and response assessment have been validated with this method. Recently, a new assay based on monoclonal antibodies (N latex FLC) has been marketed in Europe...
March 27, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28341732/novel-recurrent-chromosomal-aberrations-detected-in-clonal-plasma-cells-of-light-chain-amyloidosis-patients-show-potential-adverse-prognostic-effect-first-results-from-a-genome-wide-copy-number-array-analysis
#19
Martin Granzow, Ute Hegenbart, Katrin Hinderhofer, Dirk Hose, Anja Seckinger, Tilmann Bochtler, Kari Hemminki, Hartmut Goldschmidt, Stefan O Schönland, Anna Jauch
Immunoglobulin light chain amyloidosis is a rare plasma cell dyscrasia characterized by deposition of abnormal amyloid fibrils in multiple organs impairing their function. In the largest cohort studied up to now of 118 CD138-purified plasma cell samples from previously untreated immunoglobulin light chain amyloidosis patients, we assessed in parallel copy number alterations using high-density copy number arrays and interphase fluorescence in situ hybridization. We used fluorescence in situ hybridization probes for the IgH translocations t(11;14), t(4;14), and t(14;16) or any other IgH rearrangement as well as numerical aberrations of the chromosome loci 1q21, 8p21, 5p15/5q35, 11q22...
March 24, 2017: Haematologica
https://www.readbyqxmd.com/read/28338670/encouraging-impact-of-doxycycline-on-early-mortality-in-cardiac-light-chain-al-amyloidosis
#20
A D Wechalekar, C Whelan
No abstract text is available yet for this article.
March 24, 2017: Blood Cancer Journal
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