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Amyloidosis AL

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https://read.qxmd.com/read/30875258/zebrafish-model-of-amyloid-light-chain-cardiotoxicity-regeneration-vs-degeneration
#1
Shikha Mishra, Shaurya Joshi, Jennifer E Ward, Eva P Buys, Deepak Mishra, Deepa Mishra, Isabel Morgado, Sudeshna Fisch, Francesca Lavatelli, Giampaolo Merlini, Sharmila Dorbala, Calum A MacRae, Ronglih Liao
Cardiac dysfunction is the most frequent cause of morbidity and mortality in immunoglobulin light chain (AL) amyloidosis. Previously published transgenic animal models of AL amyloidosis have not recapitulated the key phenotype of cardiac dysfunction seen in AL amyloidosis which has limited our understanding of the disease mechanisms in vivo, as well as the development of targeted AL therapeutics. We have developed a transgenic zebrafish model in which a AL patient-derived lambda light chain (LC) is conditionally expressed in the liver under the control of UAS-Gal4 enhancer system...
March 15, 2019: American Journal of Physiology. Heart and Circulatory Physiology
https://read.qxmd.com/read/30839349/al-amyloidoma-of-the-skin-a-rare-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#2
Stefan Ueberdiek, Werner Kempf, Lutz Kretschmer, Michael Peter Schön, Christina Mitteldorf
AL-amyloidoma is considered to be a variant of primary cutaneous marginal zone lymphoma (pcMZL). A 51-year-old white man presented a 2 × 2-cm erythematous to brownish waxy plaque on the back of the scalp. The plaque was first noticed 16 years ago. It was asymptomatic, and the patient was otherwise healthy. The lesion was excised. Histological examination revealed dermal deposits of amyloid and a subtle perivascular infiltrate, predominately consisting of plasma cells. Infiltrating cells expressed CD79a, bcl2, and IgG and were negative for bcl6, CD56, and IgM...
February 25, 2019: American Journal of Dermatopathology
https://read.qxmd.com/read/30837451/two-types-of-amyloidosis-presenting-in-a-single-patient-a-case-series
#3
M Hasib Sidiqi, Ellen D McPhail, Jason D Theis, Surendra Dasari, Julie A Vrana, Maria Eleni Drosou, Nelson Leung, Suzanne Hayman, S Vincent Rajkumar, Rahma Warsame, Stephen M Ansell, Morie A Gertz, Martha Grogan, Angela Dispenzieri
The amyloidoses are a group of disorders with overlapping clinical presentations, characterized by aggregation and tissue deposition of misfolded proteins. The nature and source of the amyloidogenic protein determines therapy, therefore correct subtyping is critical to patient management. We report the clinicopathologic features of nine patients diagnosed with two amyloid types confirmed by liquid chromatography-coupled tandem mass spectrometry. The most common types were transthyrethin (n = 9) and immunoglobulin-derived (n = 7)...
March 5, 2019: Blood Cancer Journal
https://read.qxmd.com/read/30836444/clinical-features-laboratory-characteristics-and-outcomes-of-patients-with-renal-versus-cardiac-light-chain-amyloidosis
#4
Surbhi Sidana, Nidhi Tandon, Morie A Gertz, Angela Dispenzieri, Marina Ramirez-Alvarado, David L Murray, Taxiarchis V Kourelis, Francis K Buadi, Prashant Kapoor, Wilson Gonsalves, Rahma Warsame, Martha Q Lacy, Robert A Kyle, S Vincent Rajkumar, Shaji K Kumar, Nelson Leung
This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0...
March 5, 2019: British Journal of Haematology
https://read.qxmd.com/read/30820370/diagnosis-and-management-of-a-cardiac-amyloidosis-case-mimicking-hypertrophic-cardiomyopathy
#5
Yasar Sattar, Tania Ruiz Maya, Fnu Zafrullah, Nirav B Patel, Sharaad Latchana
Cardiac amyloidosis is an acquired heart disease secondary to the deposition of β-pleated amyloid proteins in heart tissue. Amyloid light chain (AL) amyloidosis is usually secondary to multiple myeloma and can rapidly deteriorate cardiac function, with high mortality. Up to 50% of AL patients have cardiac involvement presenting as heart failure, conduction abnormalities, and cardiomyopathies. One of the rare presentations is the likely simulation of disease with hypertrophic cardiomyopathies like left ventricular outflow tract (LVOT) obstruction due to the systolic anterior motion of the mitral valve and irregular septal hypertrophy secondary to amyloid deposits...
December 18, 2018: Curēus
https://read.qxmd.com/read/30816248/the-cdr1-and-other-regions-of-immunoglobulin-light-chains-are-hot-spots-for-amyloid-aggregation
#6
Robin Axel Ruiz-Zamora, Simon Guillaumé, Youssra K Al-Hilaly, Zahraa Al-Garawi, Francisco Javier Rodríguez-Alvarez, Guadalupe Zavala-Padilla, Julio I Pérez-Carreón, Sandra L Rodríguez-Ambriz, Guillermo A Herrera, Baltazar Becerril-Luján, Adrián Ochoa-Leyva, Jorge Melendez-Zajgla, Louise Serpell, Luis Del Pozo-Yauner
Immunoglobulin light chain-derived (AL) amyloidosis is a debilitating disease without known cure. Almost nothing is known about the structural factors driving the amyloidogenesis of the light chains. This study aimed to identify the fibrillogenic hotspots of the model protein 6aJL2 and in pursuing this goal, two complementary approaches were applied. One of them was based on several web-based computational tools optimized to predict fibrillogenic/aggregation-prone sequences based on different structural and biophysical properties of the polypeptide chain...
February 28, 2019: Scientific Reports
https://read.qxmd.com/read/30804262/incidence-of-pediatric-glomerular-diseases-in-arab-world-a-systematic-review
#7
REVIEW
Nasar Yousuf Alwahaibi, Halima Khalfan Al Issaei, Buthaina Saif Al Dhahli
Despite the high number of Arab children, there are a scanty available data about different types of pediatric glomerular diseases in all Arab countries. Very few Arab countries have a national renal biopsy registry. In this review, we performed a systematic review analyzing the incidence of pediatric glomerular disease in all Arab countries. Relevant manuscripts in all 22 Arab countries found through searches of Medline, Science Direct, Embase and Google Scholar were evaluated. The period was from January 1990 to March 2018...
January 2019: Saudi Journal of Kidney Diseases and Transplantation
https://read.qxmd.com/read/30803274/multiorgan-involvement-by-amyloid-light-chain-amyloidosis
#8
Guoliang Li, Dan Han, Suhua Wei, Huaiyu Wang, Limei Chen
Amyloid light chain (AL) amyloidosis is a protein conformational disease. AL amyloidosis results from aggregation of misfolded proteins that are deposited in tissues as amyloid fibrils. Diagnosis of AL amyloidosis can be challenging due to its low incidence and clinical complexity. Therapy requires a risk-adapted approach involving dose reductions and schedule modifications of chemotherapy regimens along with close monitoring of hematologic and organ responses. We herein describe a patient whose condition was diagnosed as systemic AL amyloidosis and presented with splenic rupture as the initial symptom...
February 25, 2019: Journal of International Medical Research
https://read.qxmd.com/read/30801187/classification-of-plasma-cell-disorders-by-21-tesla-fourier-transform-ion-cyclotron-resonance-top-down-and-middle-down-ms-ms-analysis-of-monoclonal-immunoglobulin-light-chains-in-human-serum
#9
Lidong He, Lissa C Anderson, David R Barnidge, David L Murray, Surendra Dasari, Angela Dispenzieri, Christopher L Hendrickson, Alan G Marshall
The current five-year survival rate for systemic AL amyloidosis or multiple myeloma is ∼51%, indicating the urgent need for better diagnosis methods and treatment plans. Here, we describe highly specific and sensitive top-down and middle-down MS/MS methods owning the advantages of fast sample preparation, ultrahigh mass accuracy, and extensive residue cleavages with 21 telsa FT-ICR MS/MS. Unlike genomic testing, which requires bone marrow aspiration and may fail to identify all monoclonal immunoglobulins produced by the body, the present method requires only a blood draw...
February 25, 2019: Analytical Chemistry
https://read.qxmd.com/read/30798427/detection-of-cardiac-amyloidosis-with-18-f-florbetaben-pet-ct-in-comparison-to-echocardiography-cardiac-mri-and-dpd-scintigraphy
#10
Malte Kircher, Sandra Ihne, Joachim Brumberg, Caroline Morbach, Stefan Knop, K Martin Kortüm, Stefan Störk, Andreas K Buck, Theresa Reiter, Wolfgang R Bauer, Constantin Lapa
PURPOSE: Cardiac amyloidosis (CA) is a rare cause of heart failure with frequently delayed diagnosis, because specific early signs or symptoms are missing. Recently, direct amyloid imaging using positron emission tomography/computed tomography (PET/CT) has emerged. The aim of this study was to examine the performance of 18 F-florbetaben-PET/CT in detection of CA, and compare it to echocardiography (echo), cardiac MRI (CMR) and scintigraphy. Additionally, the use of 18 F-florbetaben-PET/CT for quantification of amyloid burden and monitoring of treatment response was assessed...
February 23, 2019: European Journal of Nuclear Medicine and Molecular Imaging
https://read.qxmd.com/read/30796725/associations-between-health-related-quality-of-life-and-self-reported-emergency-room-department-visits-and-inpatient-hospitalizations-insights-from-a-secondary-data-analysis-of-patients-with-light-chain-al-amyloidosis
#11
Kristen L McCausland, Avery A Rizio, Michelle K White, Martha S Bayliss, Tiffany P Quock
INTRODUCTION: Light-chain (AL) amyloidosis is a rare, progressive, and typically fatal disease. Health-related quality of life (HRQoL) has been shown to be a significant prognostic factor associated with clinical outcomes such as survival and response to treatment. A better understanding of how patterns of HRQoL may be prospectively associated with costly healthcare resource utilization, such as emergency department (ED) visits and inpatient hospitalizations, is warranted. METHODS: A secondary data analysis of a non-interventional, longitudinal online study of patients with AL amyloidosis (n = 224) was conducted...
February 22, 2019: PharmacoEconomics Open
https://read.qxmd.com/read/30790171/advances-in-the-diagnosis-and-treatment-of-transthyretin-amyloidosis-with-cardiac-involvement
#12
REVIEW
Angelos G Rigopoulos, Muhammad Ali, Elena Abate, Abdel-Rahman Torky, Marios Matiakis, Mammad Mammadov, Hannes Melnyk, Alexander Vogt, Renato de Vecchis, Boris Bigalke, Walter Wohlgemuth, Sophie Mavrogeni, Michel Noutsias
Amyloidosis is caused by extracellular deposition of insoluble abnormal fibrils constituted by misfolded proteins, which can modify tissue anatomy and hinder the function of multiple organs including the heart. Amyloidosis that can affect the heart includes mostly systemic amyloidosis (amyloid light chain, AL) and transthyretin amyloidosis (ATTR). The latter can be acquired in elderly patients (ATTRwt), or be inherited in younger individuals (ATTRm). The diagnosis is demanding given the high phenotypic heterogeneity of the disease...
February 21, 2019: Heart Failure Reviews
https://read.qxmd.com/read/30773521/outcome-of-cardiac-light-chain-amyloidosis-in-the-era-of-novel-therapy-a-single-center-cohort-study-of-227-patients
#13
Jun Feng, Congli Zhang, Kaini Shen, Jian Sun, Quan Fang, Lu Zhang, Xinxin Cao, Daobin Zhou, Jian Li, Zhuang Tian
BACKGROUND: Cardiac involvement occurs in more than half of the patients with light-chain amyloidosis (AL), but the characteristics, treatment and prognosis of cardiac AL (CAL) are not fully described. Methods and Results: A total of 227 patients with CAL diagnosis between January 2009 and March 2017 at Peking Union Medical College Hospital were included. Patients with Mayo stages I, II and III AL accounted for 0.9%, 49.8% and 49.3%, respectively. Autologous stem cell transplantation, bortezomib combinations, non-bortezomib regimens and palliative treatment were given as first line therapy in 3...
February 16, 2019: Circulation Journal: Official Journal of the Japanese Circulation Society
https://read.qxmd.com/read/30771083/hypocalcemia-and-hypophosphatemia-after-treatment-with-zoledronic-acid-in-a-patient-with-al-amyloidosis
#14
Antonella Tufano, Domenico Rendina, Paolo Conca, Beniamino Matani, Giovanni Di Minno
No abstract text is available yet for this article.
February 15, 2019: Internal and Emergency Medicine
https://read.qxmd.com/read/30770096/a-modern-primer-on-light-chain-amyloidosis-in-592-patients-with-mass-spectrometry-verified-typing
#15
Eli Muchtar, Morie A Gertz, Robert A Kyle, Martha Q Lacy, David Dingli, Nelson Leung, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Yi Lisa Hwa, Amie Fonder, Miriam Hobbs, Wilson Gonsalves, Taxiarchis V Kourelis, Rahma Warsame, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, S Vincent Rajkumar, Shaji K Kumar, Angela Dispenzieri
OBJECTIVE: To describe the clinical and laboratory characteristics of patients with meticulously typed light chain (AL) amyloidosis. PATIENTS AND METHODS: Patients (N=592) with biopsy-proven, mass spectrometry-confirmed AL amyloidosis diagnosed from January 1, 2008, through August 31, 2015, were included. RESULTS: The median patient age at diagnosis was 63 years. Thirty-four percent of patients (n=204) had isolated organ involvement, mostly heart (19% [n=115]) followed by kidney (9% [n=53])...
February 13, 2019: Mayo Clinic Proceedings
https://read.qxmd.com/read/30767162/semi-quantitative-indices-of-cardiac-uptake-in-patients-with-suspected-cardiac-amyloidosis-undergoing-99mtc-hmdp-scintigraphy
#16
Chiara Gallini, Francesca Tutino, Raffaele Martone, Alfonso Ciaccio, Egidio Natalino Costanzo, Giulia Taborchi, Sofia Morini, Simone Bartolini, Silvia Farsetti, Carlo Di Mario, Federico Perfetto, Francesco Cappelli
BACKGROUND: 99mTc-HMDP scintigraphy has proved its efficacy in non-invasive diagnosis of cardiac amyloidosis (CA) and is currently interpreted according to the Perugini qualitative assessment. Several semi-quantitative indices have been proposed to overcome inherent possible limitations of visual grading. Our aim was to comparatively evaluate six different indices and their diagnostic performance. METHODS: We retrospectively reviewed scintigraphy of 76 patients (53 ATTR, 12 AL, 11 LVH) who underwent diagnostic evaluation at our centre...
February 14, 2019: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://read.qxmd.com/read/30763917/a-quercetin-based-flavanoid-rutin-reverses-amyloid-fibrillation-in-%C3%AE-lactoglobulin-at-ph-2-0-and-358-k
#17
Nasser Abdulatif Al-Shabib, Javed Masood Khan, Ajamaluddin Malik, Priyankar Sen, Mohammad A Alsenaidy, Fohad Mabood Husain, Abdulrahman M Alsenaidy, Rizwan Hasan Khan, Hani Choudhry, Mazin A Zamzami, Mohmmad Imran Khan, Syed Ali Shahzad
β-lactoglobulin (BLG) is a well characterized milk protein and a model for folding and aggregation studies. Rutin is a quercetin based-flavanoid and a famous dietary supplement. It is a potential protector from coronary heart disease, cancers, and inflammatory bowel disease. In this study, amyloid fibrillation is reported in BLG at pH 2.0 and temperature 358 K. It is inhibited to some extent by rutin with a rate of 99.3 h-1  M-1 . Amyloid fibrillation started taking place after 10 h of incubation and completed near 40 h at a rate of 16...
February 5, 2019: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
https://read.qxmd.com/read/30742933/prognostic-impact-of-light-chain-and-transthyretin-related-categories-in-cardiac-amyloidosis-a-systematic-review-and-meta-analysis
#18
Yanguo Xin, Wenyu Hu, Xin Chen, Jian Hu, Yingxian Sun, Yinan Zhao
BACKGROUND: Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant cardiac involvement. In this study, we compared prognosis in patients with different categories of cardiac amyloidosis using meta-analysis and present a systematic review. METHODS: A systematic literature search was performed through Jan 1st , 2018 and two reviewers independently extracted data and assessed risk of bias...
February 8, 2019: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://read.qxmd.com/read/30737485/prognostic-restaging-at-the-time-of-second-line-therapy-in-patients-with-al-amyloidosis
#19
Yi L Hwa, Morie A Gertz, Shaji K Kumar, Martha Q Lacy, Francis K Buadi, David Dingli, Prashant Kapoor, Steve R Zeldenrust, Nelson Leung, Susanne R Hayman, Wilson I Gonsalves, Taxiarchis V Kourelis, Rahma Warsame, Ronald S Go, Eli Muchtar, Miriam A Hobbs, Amie L Fonder, Stephen Russell, Robert A Kyle, S Vincent Rajkumar, Angela Dispenzieri
It is well known that staging of patients with AL amyloidosis at diagnosis predicts for survival, but there is a paucity of literature delineating the prognostic value of these systems at relapse. We evaluated the prognostic value of AL staging among 413 patients initiated with second-line therapy between 2000 and 2015. Both the Revised Mayo 2012 and the European revision of Mayo 2004 staging systems were used. The median time from initial treatment to second-line therapy was 11.7 months. The first-line therapy was autologous stem cell transplant (ASCT) in 179 (43%) patients and non-ASCT therapies in 234 patients...
February 8, 2019: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://read.qxmd.com/read/30718069/utility-of-the-serum-free-light-chain-assay-in-the-diagnosis-of-light-chain-amyloidosis-in-patients-with-heart-failure
#20
Ian C Chang, Angela Dispenzieri, Christopher G Scott, Grace Lin, Allan S Jaffe, Kyle W Klarich, Martha Grogan
OBJECTIVE: To determine the utility of the serum free light chain (FLC) assay for routine screening of light chain amyloidosis (AL) in patients with heart failure. PATIENTS AND METHODS: We studied consecutive new patients referred to the Heart Failure Clinic who had the FLC assay performed for routine screening at Mayo Clinic's campus in Rochester, Minnesota, from January 1, 2011, through December 31, 2015. An FLC ratio between 0.26 and 1.65 was considered normal...
February 1, 2019: Mayo Clinic Proceedings
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