Results 51 to 60 of about 5,095,980 (121)

Role of FibroScan in evaluating hepatic involvement in systemic amyloidosis: a single‐centre prospective pilot study

Internal Medicine Journal, EarlyView.
Abstract Background Amyloidosis encompasses a group of diseases characterised by extracellular deposition of misfolded protein fibrils, potentially leading to organ dysfunction. Liver involvement occurs via direct amyloid infiltration, common in immunoglobulin light chain (AL) amyloidosis, or congestive hepatopathy, more typical in transthyretin (ATTR)
M Tang, N Vootukuru, J Tjahyadi, D Clayton Chubb, H Jina, SB Ting, S Bloom, R Sawhney, S Gibbs, O. Niewiadomski   +9 more
wiley   +1 more source

Natural history and outcome in systemic AA amyloidosis [PDF]

, 2007
BACKGROUND:Deposition of amyloid fibrils derived from circulating acute-phase reactant serum amyloid A protein (SAA) causes systemic AA amyloidosis, a serious complication of many chronic inflammatory disorders.
Goodman, HJB, Lachmann, HJ, Gillmore, JD, Hawkins, PN, Gilbertson, JA, Gallimore, JR, Sabin, CA   +6 more
core  

Diagnosing Smoldering Multiple Myeloma and Light Chain Amyloidosis in the Setting of Severe Transthyretin Amyloid Cardiomyopathy and Extracardiac Sarcoidosis

Annals of Internal Medicine: Clinical Cases
Light chain (AL) amyloidosis involves deposition of monoclonal immunoglobulin light chain-derived amyloid fibrils, whereas ATTR amyloidosis involves deposition of insoluble antiparallel beta-pleated protein within tissues, leading to organ-specific ...
Robert A. Churchill, Andrew Rosenbaum
doaj   +1 more source

Localized Gastrointestinal Light Chain (AL) Amyloidosis Under Surveillance for Five Years: A Case Report

DEN Open, Volume 7, Issue 1, April 2027.
ABSTRACT Amyloidosis, characterized by the deposition of abnormal protein fibrils in organs, is classified as systemic or localized. Amyloid light chain (AL)‐type localized amyloidosis is uncommon, particularly when confined to the gastrointestinal tract.
Shunsuke Kojimahara, Keiichi Tominaga, Akira Yamamiya, Mimari Kanazawa, Takanao Tanaka, Takeshi Sugaya, Nagaaki Katoh, Atsushi Irisawa   +7 more
wiley   +1 more source

A Case of Severe Cholestasis due to Hepatic AL Amyloidosis

GE: Portuguese Journal of Gastroenterology, 2019
Introduction: Immunoglobulin light chain-associated amyloidosis results from extracellular tissue deposition of fibril-forming monoclonal immunoglobulin light chains, secreted by a clone of plasma cells.
Teresa Dias, Diana Ferreira, Hélder Moreira, Telma Nascimento, Arsénio Santos, Armando Carvalho   +5 more
doaj   +1 more source

Exploiting endogenous and therapy-induced apoptotic vulnerabilities in immunoglobulin light chain amyloidosis with BH3 mimetics

Nature Communications, 2022
Immunoglobulin light chain amyloidosis is a lethal hematologic disorder driven by clonal plasma cells producing abnormal light chains that damage healthy tissues. Fraser et al.
Cameron S. Fraser, Johan K. E. Spetz, Xingping Qin, Adam Presser, Jonathan Choiniere, Chendi Li, Stacey Yu, Frances Blevins, Aaron N. Hata, Jeffrey W. Miller, Gary A. Bradshaw, Marian Kalocsay, Vaishali Sanchorawala, Shayna Sarosiek, Kristopher A. Sarosiek   +14 more
doaj   +1 more source

Critical Management Strategies for Cardiovascular Involvementin Light Chain Amyloidosis

罕见病研究, 2023
Light chain amyloidosis (AL) is a kind of rare disease. The misfolding of the light chain of monoclonal immunoglobulin forms amyloid substances and deposit in different tissues and organs, resulting in organ dysfunction.
SHI Yunjing, JIN Wei
doaj   +1 more source

Aggressive Hepatic AL Amyloidosis Encountering Therapeutic Frailty: Rapid Organ Reversal With Tailored Daratumumab‐Based Therapy and Low‐Dose Corticosteroids in a Patient With Steroid‐Induced Psychosis

Clinical Case Reports, Volume 14, Issue 11, November 2026.
ABSTRACT Hepatic AL amyloidosis can cause severe cholestasis independent of marrow tumor burden. In patients with absolute contraindications to high‐dose steroids—such as steroid‐induced psychosis—tailored daratumumab regimens with low‐dose corticosteroids can drive rapid, life‐saving organ reversal.
R. Ceccarelli, M. Offidani, E. Morsia, A. Poloni, A. Mandolesi, G. Goteri, M. Valente, A. Ranghino, S. Morè   +8 more
wiley   +1 more source

Do not forget to check the scalp in systemic light-chain amyloidosis [PDF]

, 2021
Systemic light chain amyloidosis (AL) is a nonproliferative plasma cell disorder in which fragments of immunoglobulin deposit in tissues. Clinical manifestations are often nonspecific, making diagnosis challenging.Biopsy of involved organs is the gold ...
Larrondo, Jorge, Cabrera, Raúl, Jeraldo, Cecilia, McMichael, Amy, Castro, Alex, Gosch, Marianne, Pena, Camila   +6 more
core   +1 more source

Light Chain Monoclonal Gammopathy of Undetermined Significance: Diagnosis, Biology, and Clinical Management

European Journal of Haematology, Volume 117, Issue 5, Page 1159-1167, November 2026.
ABSTRACT Light chain monoclonal gammopathy of undetermined significance (LC‐MGUS) is defined by an abnormal serum free light chain ratio and elevated involved light chain in the absence of a detectable immunoglobulin heavy chain on immunofixation and of end‐organ damage attributable to a plasma cell disorder.
Sigurður Yngvi Kristinsson, Thórir Einarsson Long, Sigrún Thorsteinsdóttir   +2 more
wiley   +1 more source