Results 1 to 10 of about 461,541 (350)

Immunoglobulin light chain amyloidosis diagnosis and treatment algorithm 2021 [PDF]

Blood Cancer Journal, 2021
Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients ...
M. Hasib Sidiqi, Morie A. Gertz
doaj   +3 more sources

Systemic immunoglobulin light chain amyloidosis [PDF]

Nature Reviews Disease Primers, 2018
Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction.
G. Merlini, A. Dispenzieri, V. Sanchorawala, S. Schönland, G. Palladini, P. Hawkins, M. Gertz   +6 more
semanticscholar   +7 more sources

99mTc-DPD scintigraphy in immunoglobulin light chain (AL) cardiac amyloidosis. [PDF]

Eur Heart J Cardiovasc Imaging, 2021
Aims Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD scintigraphy) is recognized as highly accurate for the non-invasive diagnosis of transthyretin (ATTR) cardiac amyloidosis (CA).
Quarta CC, Zheng J, Hutt D, Grigore SF, Manwani R, Sachchithanantham S, Mahmood SA, Whelan CJ, Fontana M, Martinez-Naharro A, Chacko L, Lachmann HJ, Gillmore JD, Rapezzi C, Hawkins PN, Wechalekar AD.   +15 more
europepmc   +2 more sources

Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis. [PDF]

New England Journal of Medicine, 2021
BACKGROUND Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by deposition of amyloid fibrils of light chains produced by clonal CD38+ plasma cells. Daratumumab, a human CD38-targeting antibody, may improve outcomes for this disease.
E. Kastritis, G. Palladini, M. Minnema, A. Wechalekar, A. Jaccard, Hans C. Lee, V. Sanchorawala, S. Gibbs, P. Mollee, C. Venner, Jin-Hai Lu, S. Schönland, M. Gatt, Kenshi Suzuki, Kihyun Kim, M. Cibeira, M. Beksaç, E. Libby, J. Valent, V. Hungria, S. Wong, M. Rosenzweig, N. Bumma, A. Huart, M. Dimopoulos, D. Bhutani, A. Waxman, S. Goodman, J. Zonder, Selay Lam, K. Song, T. Hansen, S. Manier, W. Roeloffzen, K. Jamroziak, F. Kwok, C. Shimazaki, Jin-Seok Kim, E. Crusoé, T. Ahmadi, N. Tran, X. Qin, S. Vasey, B. Tromp, J. Schecter, B. Weiss, S. Zhuang, J. Vermeulen, G. Merlini, R. Comenzo   +49 more
semanticscholar   +12 more sources

Immunoglobulin light chain amyloid aggregation. [PDF]

Chemical Communications, 2018
Light chain (AL) amyloidosis is a devastating, complex, and incurable protein misfolding disease. It is characterized by an abnormal proliferation of plasma cells (fully differentiated B cells) producing an excess of monoclonal immunoglobulin light ...
L. Blancas-Mejía, Pinaki Misra, C. Dick, Shawna A. Cooper, Keely R. Redhage, Michael R. Bergman, Torri L. Jordan, Khansaa Maar, M. Ramirez-Alvarado   +8 more
semanticscholar   +4 more sources

Machine learning analyses of antibody somatic mutations predict immunoglobulin light chain toxicity. [PDF]

Nat Commun, 2021
In systemic light chain amyloidosis (AL), pathogenic monoclonal immunoglobulin light chains (LC) form toxic aggregates and amyloid fibrils in target organs.
Garofalo M, Piccoli L, Romeo M, Barzago MM, Ravasio S, Foglierini M, Matkovic M, Sgrignani J, De Gasparo R, Prunotto M, Varani L, Diomede L, Michielin O, Lanzavecchia A, Cavalli A.   +14 more
europepmc   +2 more sources

Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications [PDF]

Experimental Hematology & Oncology, 2021
Immunoglobulin light chain amyloidosis (AL) is an indolent plasma cell disorder characterized by free immunoglobulin light chain (FLC) misfolding and amyloid fibril deposition.
Linchun Xu, Yongzhong Su
doaj   +2 more sources

Germline polymorphisms and alternative splicing of human immunoglobulin light chain genes [PDF]

iScience, 2021
Summary: Inference of germline polymorphisms in immunoglobulin genes from B cell receptor repertoires is complicated by somatic hypermutations, sequencing/PCR errors, and by varying length of reference alleles.
Ivana Mikocziova, Ayelet Peres, Moriah Gidoni, Victor Greiff, Gur Yaari, Ludvig M. Sollid   +5 more
doaj   +2 more sources

Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve [PDF]

Case Reports in Neurology, 2021
Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various ...
Shinsuke Morisaki, Shinji Tsuchida, Eiichi Konishi, Nagaaki Katoh, Yusuke Takahashi, Kenji Takahashi   +5 more
doaj   +2 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