Results 21 to 30 of about 17,175,483 (375)

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

Guidelines for non-transplant chemotherapy for treatment of systemic AL amyloidosis: EHA-ISA working group

Amyloid: Journal of Protein Folding Disorders, 2022
Background This guideline has been developed jointly by the European Society of Haematology and International Society of Amyloidosis recommending non-transplant chemotherapy treatment for patients with AL amyloidosis.
A. Wechalekar, M. Cibeira, S. Gibbs, A. Jaccard, Shaji K. Kumar, G. Merlini, G. Palladini, V. Sanchorawala, S. Schönland, C. Venner, M. Boccadoro, E. Kastritis   +11 more
semanticscholar   +1 more source

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Blood Cancer Journal, 2021
The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival
Andrew Staron, Luke Zheng, G. Doros, L. Connors, L. Mendelson, T. Joshi, V. Sanchorawala   +6 more
semanticscholar   +1 more source

Daratumumab in AL amyloidosis

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
openaire   +3 more sources

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

Systemic Immunoglobulin Light Chain Amyloidosis Presenting as Painful Penile Ulcers

Annals of Internal Medicine: Clinical Cases, 2022
Immunoglobulin light chain (AL) amyloidosis is a systemic form of amyloidosis that commonly affects the heart, kidney, liver, and nervous system. Penile involvement in AL amyloidosis is rare and thus often not considered in the differential diagnosis for
Crystal Lihong Yan, Fireneh N. Beshah, Karen Eliahu, James E. Hoffman   +3 more
doaj   +1 more source

Prognostic importance of the 6 min walk test in light chain (AL) amyloidosis

Heart, 2022
Objectives In AL amyloidosis, organ response assessment is based on surrogates (eg, cardiac biomarkers). An objective functional test, such as the 6 min walk test (6MWT), capturing overall clinical improvement, is required.
O. Cohen, Ananth Sathyanath, A. Petrie, S. Ravichandran, S. Law, R. Manwani, Darren Foard, S. Sachchithanantham, S. Mahmood, A. Martinez-Naharro, M. Fontana, C. Whelan, P. Hawkins, H. Lachmann, J. Gillmore, A. Wechalekar   +15 more
semanticscholar   +1 more source

High response rates with single-agent belantamab mafodotin in relapsed systemic AL amyloidosis

Blood Cancer Journal, 2022
Dear editor, Systemic amyloid light-chain (AL) amyloidosis is a rare plasma cell disorder caused by the extracellular deposition of misfolded immunoglobulin light chains as protein fibrils in tissues. This leads to vital organ damage. It is incurable and
J. Khwaja, Joshua Bomsztyk, S. Mahmood, Brendan Wisniowski, R. Shah, A. Tailor, K. Yong, R. Popat, N. Rabin, C. Kyriakou, J. Sive, S. Esposti, D. Larkin, S. Worthington, Alyse Hart, Emma Dowling, Nuno Correia, Ceri A Bygrave, A. Rydzewski, K. Jamroziak, A. Wechalekar   +20 more
semanticscholar   +1 more source

Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis

International Journal of Molecular Sciences, 2022
In amyloid light-chain (AL) amyloidosis, small B-cell clones (mostly plasma cell clones) present in the bone marrow proliferate and secrete unstable monoclonal free light chains (FLCs), which form amyloid fibrils that deposit in the interstitial tissue ...
Hidehiko Ikura, J. Endo, Hiroki Kitakata, Hidenori Moriyama, M. Sano, K. Fukuda   +5 more
semanticscholar   +1 more source

Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study [PDF]

, 2020
Background Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities.
Coelho, Teresa, Conceição, Isabel, Costa, João, de Carvalho, Mamede, Ferreira, Lara, Inês, Mónica   +5 more
core   +2 more sources