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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 ...
Booth, D.R., Booth, S.E., Bybee, A., Gilbertson, J.A., Gillmore, J.D., Hawkins, P.N., Lachmann, H.J., Pepys, M.B.   +7 more
core   +7 more sources

AL Amyloidosis [PDF]

Orphanet Journal of Rare Diseases, 2012
Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone.
Desport Estelle, Bridoux Frank, Sirac Christophe, Delbes Sébastien, Bender Sébastien, Fernandez Béatrice, Quellard Nathalie, Lacombe Corinne, Goujon Jean-Michel, Lavergne David, Abraham Julie, Touchard Guy, Fermand Jean-Paul, Jaccard Arnaud   +13 more
doaj   +8 more sources

Expert recommendation from the Swiss Amyloidosis Network (SAN) for systemic AL-amyloidosis

Swiss Medical Weekly, 2020
Systemic amyloidosis is a heterogeneous group of diseases associated with protein misfolding into insoluble beta-sheet rich structures that deposit extracellularly in different organs, eventually compromising their function.
Rahel Schwotzer, Andreas J. Flammer, Sabine Gerull, Thomas Pabst, Paolo Arosio, Manuela Averaimo, Ulrike Bacher, Peter Bode, Andrea Cavalli, Adalgisa Condoluci, Stefan Dirnhofer, Nadia Djerbi, Stephan W. Dobner, Thomas Fehr, Maura Garofalo, Ariana Gaspert, Raphael Heimgartner, Annemarie Hübers, Hans H. Jung, Chiara Kessler, Raphael Knöpfel, Natallia Laptseva, Robert Manka, Luca Mazzucchelli, Martin Meyer, Violeta Mihaylova, Pierre Monney, Alessio Mylonas, René Nkoulou, Aju P. Pazhenkottil, Otmar Pfister, Axel Rüfer, Adrian Schmidt, Harald Seeger, Simon F. Stämpfli, Guido Stirnimann, Thomas Suter, Marie Théaudin, Giorgio Treglia, Alexandar Tzankov, Friederike Vetter, Markus Zweier, Bernhard Gerber   +42 more
doaj   +2 more sources

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

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   +2 more sources

Chronic Viral Infections and Al Amyloidosis: An Uncommon Association [PDF]

Clinical Case Reports
AL‐amyloidosis is caused by deposition of pathological insoluble proteins in organs and tissues. HIV infection is a multi‐system disorder causing chronic inflammation.
Alberto Palladini, Gabriele Cusumano, Ottavio Martellucci, Antonietta Gigante, Gabriella D'Ettorre, Maria Teresa Petrucci, Maurizio Muscaritoli, Mariangela Palladino   +7 more
doaj   +2 more sources

A RARE PRESENTATION OF SYSTEMIC AL AMYLOIDOSIS; PULMONARY AL AMYLOIDOSIS

Hematology, Transfusion and Cell Therapy, 2022
Objective: Involvement of the lung is common in systemic AL amyloidosis in post-mortem series. However, the diagnosis is challenging. Histology is the gold standard but may result in bleeding. Consequently, diagnosis during life is rare.
Simge ERDEM, Nigar TAGHİYEVA, Kaan AKIN, Hilal KONYAOĞLU, Sevgi KALAYOĞLU BEŞIŞIK   +4 more
doaj   +2 more sources

Biomarkers in AL Amyloidosis [PDF]

International Journal of Molecular Sciences, 2021
Systemic AL amyloidosis is a rare complex hematological disorder caused by clonal plasma cells which produce amyloidogenic immunoglobulins. Outcome and prognosis is the combinatory result of the extent and pattern of organ involvement secondary to amyloid fibril deposition and the biology and burden of the underlying plasma cell clone.
Despina Fotiou, Foteini Theodorakakou, Efstathios Kastritis   +2 more
openaire   +3 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

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

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