Results 1 to 10 of about 11,291,129 (270)

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

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   +2 more sources

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

New England Journal of Medicine, 2002
Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A alpha-chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential diagnosis of systemic amyloidosis unless there is a family history.We studied 350 patients with systemic ...
Lachmann, H.J., Booth, D.R., Booth, S.E., Bybee, A., Gilbertson, J.A., Gillmore, J.D., Pepys, M.B., Hawkins, P.N.   +7 more
openaire   +5 more sources

Daratumumab in AL amyloidosis [PDF]

Blood, 2022
Abstract Light-chain amyloidosis has come far, with the first treatment getting regulatory approval in 2021. Daratumumab-based regimens achieve deep hematologic and organ responses, offering a new therapeutic backbone. Early identification, correct fibril typing, challenges of the very advanced patient, and lack of therapies to remove ...
Ashutosh D. Wechalekar, Vaishali Sanchorawala   +1 more
core   +5 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

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

Systemic amyloidosis journey from diagnosis to outcomes: a twelve-year real-world experience of a single center in a middle-income country

Orphanet Journal of Rare Diseases, 2022
Background Systemic amyloidosis is caused by the deposition of misfolded protein aggregates in tissues, leading to progressive organ dysfunction and death.
Roberta Shcolnik Szor, Fabio Fernandes, Angelina Maria Martins Lino, Leonardo Oliveira Mendonça, Fernanda Salles Seguro, Valkercyo Araujo Feitosa, Jussara Bianchi Castelli, Lecticia Barbosa Jorge, Lucas Bassolli de Oliveira Alves, Precil Diego Miranda de Menezes Neves, Evandro de Oliveira Souza, Livia Barreira Cavalcante, Denise Malheiros, Jorge Kalil, Gracia Aparecida Martinez, Vanderson Rocha   +15 more
doaj   +1 more source

Potential clinical relevance of cardiac magnetic resonance to diagnose cardiac light chain amyloidosis.

PLoS ONE, 2022
BackgroundWhile patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging.
Zsofia Dohy, Liliana Szabo, Zoltan Pozsonyi, Ibolya Csecs, Attila Toth, Ferenc Imre Suhai, Csilla Czimbalmos, Andrea Szucs, Anna Reka Kiss, David Becker, Bela Merkely, Hajnalka Vago   +11 more
doaj   +1 more source

AL amyloidosis enhances development of amyloid A amyloidosis [PDF]

British Journal of Dermatology, 2007
Contains fulltext : 52125.pdf (Publisher’s version ) (Open Access)
Hilst, J.C.H. van der, Meer, J.W.M. van der, Drenth, J.P.H., Simon, A.   +3 more
openaire   +1 more source