Results 11 to 20 of about 17,175,483 (375)

Perspectives in treatment of AL amyloidosis [PDF]

British Journal of Haematology, 2007
SummaryLight chain (AL) amyloidosis is the most frequently diagnosed form of systemic amyloid in the western world. The historically poor prognosis of AL amyloidosis appears to be improving with currently reported median survival of c. 40 months compared to 13 months in the early 1990s when low‐dose oral melphalan was the mainstay of treatment ...
Ashutosh D. Wechalekar, Philip N. Hawkins, Julian D. Gillmore   +2 more
openaire   +4 more sources

Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient

Nature Communications, 2019
Immunoglobulin Light Chain Amyloidosis (AL) is the most common systemic amyloidosis occurring in Western countries. Here the authors present the 4.0 Å cryo-EM structure of light chain AL55 fibrils that were isolated from the heart of an AL systemic ...
Paolo Swuec, Francesca Lavatelli, Masayoshi Tasaki, Cristina Paissoni, Paola Rognoni, Martina Maritan, Francesca Brambilla, Paolo Milani, Pierluigi Mauri, Carlo Camilloni, Giovanni Palladini, Giampaolo Merlini, Stefano Ricagno, Martino Bolognesi   +13 more
doaj   +2 more sources

IgM-Related Immunoglobulin Light Chain (AL) Amyloidosis

Hemato, 2022
Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic disorder characterized by an IgM paraprotein. The clinical presentation of WM varies and can include common manifestations such as anemia and hyperviscosity, in addition to less common ...
Shayna Sarosiek, Andrew R. Branagan, Steven P. Treon, Jorge J. Castillo   +3 more
doaj   +1 more source

The management of light chain (AL) amyloidosis in Europe: clinical characteristics, treatment patterns, and efficacy outcomes between 2004 and 2018

Blood Cancer Journal, 2023
Systemic light-chain (AL) amyloidosis is a rare and debilitating disease. Advances have been made in new treatments in recent years, yet real-world data on the management of the disease are scarce.
G. Palladini, S. Schönland, G. Merlini, P. Milani, A. Jaccard, F. Bridoux, M. Dimopoulos, S. Ravichandran, U. Hegenbart, W. Roeloffzen, M. Cibeira, H. Agis, M. Minnema, R. Bergantim, R. Hájek, C. João, A. Leonidakis, G. Cheliotis, P. Sonneveld, E. Kastritis, A. Wechalekar   +20 more
semanticscholar   +1 more source

Biopsy Evidence of Sequential Transthyretin and Immunoglobulin Light-Chain Cardiac Amyloidosis in the Same Patient

JACC: Case Reports, 2021
Currently adopted diagnostic flow charts consider transthyretin and light-chain cardiac amyloidosis as mutually exclusive. Here, we report for the first time, to our knowledge, the demonstration of a biopsy-proven dual pathology in an 80-year-old man ...
Giuseppe Vergaro, MD, PhD, Vincenzo Castiglione, MD, Roberta Poletti, MD, Gabriele Buda, MD, Angela Pucci, MD, PhD, Veronica Musetti, BSc, Dario Genovesi, MD, Alberto Aimo, MD, Claudio Passino, MD, Michele Emdin, MD, PhD   +9 more
doaj   +1 more source

Teclistamab in relapsed or refractory AL amyloidosis, a multinational retrospective case series.

Blood, 2023
Teclistamab shows promising results in R/R AL amyloidosis, with VGPR or better in 88% of patients and involved FLC < 10 mg/L in 76%. We observed a 35% rate of severe infections and no cardiac or kidney related events.
Nathalie Forgeard, D. Elessa, A. Carpinteiro, Karim Belhadj, Monique C. Minnema, M. Roussel, A. Huart, V. Javaugue, Laurent Pascal, B. Royer, Alexis Talbot, R. Gounot, U. Hegenbart, S. Schonland, L. Karlin, S. Harel, E. Kastritis, F. Bridoux, A. Jaccard, B. Arnulf   +19 more
semanticscholar   +1 more source

Systemic AL amyloidosis: current approach and future direction

OncoTarget, 2023
Systemic Light chain (AL) amyloidosis is a monoclonal plasma cell proliferative disorder characterized by deposition of amyloidogenic monoclonal light chain fragments causing organ dysfunction. It is a fatal disease and if not diagnosed and treated early
M. Bou Zerdan, Lewis Nasr, F. Khalid, Sabine Allam, Y. Bouferraa, Saba Batool, Muhammad Tayyeb, Shubham Adroja, Mahinbanu Mammadii, F. Anwer, S. Raza, C. Chaulagain   +11 more
semanticscholar   +1 more source

Diagnosis for Chinese patients with light chain amyloidosis: a scoping review

Annals of Medicine, 2023
Background Amyloid light chain (AL) amyloidosis is the most common systemic amyloidosis. The objective of this scoping review was to map the available literature on the diagnosis of AL amyloidosis in China.Materials and Methods The published academic ...
Meilan Chen, Junru Liu, Xiaohong Wang, Xian Cao, Xin Gao, Lingjie Xu, Wang Liu, Jingnan Pi, Bin Wang, Juan Li   +9 more
doaj   +1 more source

Anti-B Cell Maturation Antigen Chimeric Antigen Receptor T Cell Therapy for the Treatment of AL Amyloidosis and Concurrent Relapsed/Refractory Multiple Myeloma: Preliminary Efficacy and Safety

Current Oncology, 2023
While immunotherapies, such as CAR T therapy and bi-specific antibodies, have revolutionized the treatment of multiple myeloma (MM), patients with AL amyloidosis have been excluded from trials with these agents due to concerns of underlying autonomic ...
Saurav Das, S. Ailawadhi, T. Sher, Vivek Roy, Andre Fernandez, Ricardo D. Parrondo   +5 more
semanticscholar   +1 more source

Predictors of treatment response and survival outcomes in patients with advanced cardiac AL amyloidosis

Blood Advances, 2023
Key Points • We identified baseline factors predictive of treatment outcomes and prognostic for survival in stage IIIb AL amyloidosis.• Early hematologic and cardiac responses during treatment predict longer survival in stage IIIb AL amyloidosis.
J. Gustine, Andrew Staron, L. Mendelson, T. Joshi, D. Gopal, F. Ruberg, O. Siddiqi, V. Sanchorawala   +7 more
semanticscholar   +1 more source