Amyloid light-chain amyloidosis (AL) is characterized by deposits of immunoglobulin 魏 or 位 free light chains (FLCs) produced by clonal bone marrow plasma cells. FLCs transform into amyloid fibrils and accumulate progressively in multiple organs and tissues. The main targets are the heart and ...
Immunoglobulin light chain amyloidosis (AL) is the most common form of systemic amyloidosis, accounting for about 70% of all cases [34]. This disease occurs typically in people with monoclonal gammopathy, a disorder characterized by proliferation of the plasma cell clones. The increased Ig-LC prod...
Amyloid light chain (AL) amyloidosis, Congo red, chemotherapy, splenic rupture, amyloid deposition, multiorgan involvementAmyloid light chain (AL) amyloidosis... G Li,D Han,S Wei,... - 《Journal of International Medical Research》 被引量: 0发表: 2019年 Advancing precision medicine in immunoglob...
Systemic light chain (AL) amyloidosis is caused byBlood: The Journal of the American Society of HematologyOliva L, Orfanelli U, Resnati M, et al. The amyloidogenic light chain is a stressor that sensitizes plasma cells to proteasome inhibitor toxicity. Blood. 2017;129(15):2132-2142. https...
Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in target organs. Due to genetic rearrangement and somatic hypermutation, virtually, each AL patient presents a different amyloidogenic ...
Primary amyloid light-chain (AL) amyloidosis is the most frequent type, with nearly 50% of all patients developing cardiac involvement [1]. It has been reported that the median survival of the patients is 6 months from the onset of heart failure (HF) if untreated [2], while patients who...
To assess the feasibility and prognostic value of minimal residual disease (MRD) evaluated by multiparameter flow cytometry (MFC) in newly diagnosed amyloid light chain (AL) amyloidosis. Methods Clinical data from 25 consecutive newly diagnosed AL amyloidosis patients with MRD data tested at 3 months...
Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here we report the structure of a λ1 AL amyloid
amyloids. Glenner and his colleagues soon applied the method to the “primary” (today: AL) amyloidosis [48] and found the relationship between this amyloidosis and immunoglobulin light chains. AL amyloidosis is a neoplastic disease and belongs to the clinically most significant amyloid -related ...
Amyloid light-chain (AL) amyloidosis is a systemic disease characterized by the deposition of misfolded immunoglobulin light chain, causing organ failure, and in particular cardiac involvement is a leading cause of morbidity and mortality. We report the case of a 47-year-old man without prior card...