Results 41 to 50 of about 16,704,778 (257)

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

How I Treat AL Amyloidosis.

Blood, 2021
The treatment of patients with systemic light chain (AL) amyloidosis is a challenge to hematologists. Despite its generally small size, the underlying clone causes a rapidly progressing, often devastating multiorgan dysfunction through the toxic light ...
G. Palladini, G. Merlini
semanticscholar   +1 more source

Minimal residual disease negativity by next-generation flow cytometry is associated with improved organ response in AL amyloidosis

Blood Cancer Journal, 2021
Light chain (AL) amyloidosis is caused by a small B-cell clone producing light chains that form amyloid deposits and cause organ dysfunction. Chemotherapy aims at suppressing the production of the toxic light chain (LC) and restore organ function ...
G. Palladini, B. Paiva, A. Wechalekar, M. Massa, P. Milani, M. Lasa, S. Ravichandran, I. Krsnik, M. Basset, L. Burgos, M. Nuvolone, R. Lecumberri, A. Foli, N. Puig, M. Sesta, M. Bozzola, Pasquale Cascino, Alice Nevone, Jessica Ripepi, P. Berti, Simona Casarini, O. Annibali, A. Órfão, J. San-Miguel, G. Merlini   +24 more
semanticscholar   +1 more source

Amyloidosis – short review

Journal of Medical Science, 2016
Amyloidosis is a heterogeneous group of disorders associated with pathological deposition of amyloid. We can recognize two major categories of amyloidosis: primary (AL) and secondary (AA) type.
Agnieszka Danuta Gaczkowska, Paweł Jagodziński, Adrianna Mostowska   +2 more
doaj   +1 more source

Evaluation of peripheral amyloid neuropathy

Неврология, нейропсихиатрия, психосоматика, 2021
Symptoms of peripheral nervous system (PNS) damage are common clinical manifestations of systemic amyloidosis. Peripheral amyloid neuropathy is characterized by a progressive course, leading to the disability of patients; however, the current ...
O. E. Zinovyeva, E. I. Safiulina, N. S. Shcheglova, Z. V. Surnina, A. M. Nosovskiy   +4 more
doaj   +1 more source

Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis.

European Heart Journal, 2021
AIMS Cardiac involvement, a major determinant of prognosis in AL (light-chain immunoglobulin) amyloidosis, is characterized by an impairment of longitudinal strain (LS%).
O. Cohen, A. Ismael, B. Pawarova, R. Manwani, S. Ravichandran, S. Law, Darren Foard, A. Petrie, Sevda Ward, Brooke Douglas, A. Martinez-Naharro, L. Chacko, C. Quarta, S. Mahmood, S. Sachchithanantham, H. Lachmann, P. Hawkins, J. Gillmore, M. Fontana, R. Falk, C. Whelan, A. Wechalekar   +21 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  

Purpuric halo around hemangioma as a clue for primary systemic amyloidosis: Case report and review of literature

Dermatologica Sinica, 2018
Mucocutaneous lesion appears in up to 40% of patients with primary systemic amyloidosis (AL amyloidosis). The cutaneous signs may be co-expressed with other organ involvement or even solely presented as the first clue.
Yin-Shuo Chang, Kuo-Hsien Wang, Hsiou-Hsin Tsai, Chung-Hong Hu, Jan-Show Chu   +4 more
doaj   +1 more source

Daratumumab in the Treatment of Light-Chain (AL) Amyloidosis

Cells, 2021
Systemic light-chain (AL) amyloidosis is caused by a small B cell, most commonly a plasma cell (PC), clone that produces toxic light chains (LC) that cause organ dysfunction and deposits in tissues.
G. Palladini, P. Milani, F. Malavasi, G. Merlini   +3 more
semanticscholar   +1 more source

Duodenal biopsy: an unexpected confirmatory test for a patient with al-amyloidosis

Sri Lanka Journal of Medicine, 2019
SSystemic AL- amyloidosis is a disorder of protein folding in which there is extra-cellular accumulation as β pleated fibrillar deposits of monoclonal immunoglobulin light chain fragments. AL- amyloidosis is a rare clinical entity.
S. Pirasath, T. Kumanan, G. Selvaratnam, V. Sujanitha, D. D. Dikowita   +4 more
doaj   +1 more source