Results 1 to 10 of about 159,323 (346)

Disease burden of ATTR amyloidosis based on the SF-36® health survey [PDF]

Cardio-Oncology
Transthyretin (ATTR) amyloidosis is a progressive systemic disease associated with substantial morbidity and mortality. Although health-related quality of life (HRQoL) assessments are increasingly incorporated into clinical trials, data in ATTR ...
Vaishali Sanchorawala, Alexandra Greatsinger, Andrew Staron, John L. Berk, Lisa M. Mendelson, Tracy Joshi, Natasha J. Burke, Nadia Tabatabaeepour, Jess Smith, James Signorovitch   +9 more
doaj   +2 more sources

Misdiagnosis of Hereditary Amyloidosis as AL (Primary) Amyloidosis [PDF]

New England Journal of Medicine, 2002
Background: Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A -chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential ...
David R Booth, Julian D Gillmore, Mark B Pepys   +2 more
exaly   +1 more source

Impact of early response on outcomes in AL amyloidosis following treatment with frontline Bortezomib

Blood Cancer Journal, 2021
The outcomes in systemic AL amyloidosis are dependent on the depth of haematologic response. However, there is limited data on the impact of the speed of response on outcomes.
Sriram Ravichandran, Oliver C. Cohen, Steven Law, Darren Foard, Marianna Fontana, Ana Martinez-Naharro, Carol Whelan, Julian D. Gillmore, Helen J. Lachmann, Sajitha Sachchithanantham, Shameem Mahmood, Philip N. Hawkins, Ashutosh D. Wechalekar   +12 more
doaj   +1 more source

Combined Subcutaneous Fat Aspirate and Skin Tru-Cut Biopsy for Amyloid Screening in Patients with Suspected Systemic Amyloidosis

Molecules, 2021
Screening for systemic amyloidosis is typically carried out with abdominal fat aspirates with varying reported sensitivities. Fat aspirates are preferred for use in primary screening instead of organ biopsies as they are less invasive and thereby ...
Charlotte Toftmann Hansen, Hanne E. H. Møller, Aleksandra Maria Rojek, Niels Marcussen, Hans Christian Beck, Niels Abildgaard   +5 more
doaj   +1 more source

Cardiac biomarkers are prognostic in systemic light chain amyloidosis with no cardiac involvement by standard criteria

Haematologica, 2020
Patients with systemic immunoglobulin light chain amyloidosis (AL) with no evidence of cardiac involvement by consensus criteria have excellent survival, but 20% will die within 5 years of diagnosis and prognostic factors remain poorly characterised.
Faye A. Sharpley, Marianna Fontana, Ana Martinez-Naharro, Richa Manwani, Shameem Mahmood, Sajitha Sachchithanantham, Helen J. Lachmann, Julian D. Gillmore, Carol J. Whelan, Philip N. Hawkins, Ashutosh D. Wechalekar   +10 more
doaj   +1 more source

First third-generation CAR T cell application targeting CD19 for the treatment of systemic IgM AL amyloidosis with underlying marginal zone lymphoma

Biomarker Research, 2023
Light chain amyloidosis (AL) is a rare disease caused by the generalized deposition of misfolded free light chains. Patients with immunoglobulin M gammopathy (IgM) and indolent B-cell lymphoma such as marginal zone lymphoma (MZL) may in some instances ...
Felix Korell, Stefan Schönland, Anita Schmitt, Madelaine Jansen, Kiavasch Farid, Carsten Müller-Tidow, Peter Dreger, Michael Schmitt, Ute Hegenbart   +8 more
doaj   +1 more source

Amyloidosis [PDF]

Postgraduate Medical Journal, 2001
Abstract Amyloidosis is not a single disease but a series of diseases in which there is extracellular deposition of a protein which, although it may be derived from different and unrelated sources, folds into a β pleated sheet. There have recently been significant advances in elucidating the pathogenesis and in the ...
M F, Khan, R H, Falk
openaire   +2 more sources

Paraskeletal and extramedullary plasmacytomas in multiple myeloma at diagnosis and at first relapse: 50-years of experience from an academic institution

Blood Cancer Journal, 2022
From January 1970 to December 2018, 1304 patients were diagnosed with multiple myeloma (MM) at our institution and 256 (19.6%) had plasmacytomas (Ps) (paraskeletal –PPs- 17.6%, extramedullary –EMPs-1.9%).
Raquel Jiménez-Segura, Laura Rosiñol, Ma Teresa Cibeira, Carlos Fernández de Larrea, Natalia Tovar, Luis Gerardo Rodríguez-Lobato, Esther Bladé, David F. Moreno, Aina Oliver-Caldés, Joan Bladé   +9 more
doaj   +1 more source

Arrhythmic Burden in Cardiac Amyloidosis: What We Know and What We Do Not

Biomedicines, 2022
Cardiac amyloidosis (CA), caused by the deposition of insoluble amyloid fibrils, impairs different cardiac structures, altering not only left ventricle (LV) systo-diastolic function but also atrial function and the conduction system.
Alessia Argirò, Annamaria Del Franco, Carlotta Mazzoni, Marco Allinovi, Alessia Tomberli, Roberto Tarquini, Carlo Di Mario, Federico Perfetto, Francesco Cappelli, Mattia Zampieri   +9 more
doaj   +1 more source

Disease progression in cardiac transthyretin amyloidosis is indicated by serial calculation of National Amyloidosis Centre transthyretin amyloidosis stage

ESC Heart Failure, 2020
Aims Cardiac transthyretin amyloidosis (ATTR‐CM) is a progressive and fatal condition. Prognosis can be determined at diagnosis according to the National Amyloidosis Centre (NAC) transthyretin amyloidosis (ATTR) stage.
Steven Law, Aviva Petrie, Liza Chacko, Oliver C. Cohen, Sriram Ravichandran, Janet A. Gilbertson, Dorota Rowczenio, Ashutosh Wechalekar, Ana Martinez‐Naharro, Helen J. Lachmann, Carol J. Whelan, David F. Hutt, Philip N. Hawkins, Marianna Fontana, Julian D. Gillmore   +14 more
doaj   +1 more source