Results 21 to 30 of about 19,748 (182)
How I manage systemic light chain amyloidosis
Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient.
Gregory P. Kaufman, Claudio Cerchione
doaj +1 more source
Biomarkers in Immunoglobulin Light Chain Amyloidosis
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction.
Kufova, Z. +17 more
openaire +4 more sources
Immunoglobulin light chain amyloidosis [PDF]
SummaryImmunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed life-threatening systemic disease, primarily caused by insoluble depositions of misfolded monoclonal light chains. The monoclonal light chain paraprotein originates from a small clonal B‑cell or a clonal plasma cell population.
Hermine Agis, Maria T. Krauth
openaire +1 more source
Systemic Light Chain Amyloidosis
Systemic light chain (AL) amyloidosis is a rare protein misfolding and deposition disorder. Clonal plasma cells or rarely B cells produce immunoglobulin light chains with the potential to misfold. Treatment is clone directed with the goal to achieve a complete or at least very good hematological remission.
Maurizio, Marvisi, Sara, Ramponi
+8 more sources
Immunoglobulin Light Chain Amyloidosis (AL) is the most common systemic amyloidosis occurring in Western countries. Here the authors present the 4.0 Å cryo-EM structure of light chain AL55 fibrils that were isolated from the heart of an AL systemic ...
Paolo Swuec +13 more
doaj +1 more source
Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve
Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various ...
Shinsuke Morisaki +5 more
doaj +1 more source
Idiopathic membranous nephropathy with renal amyloidosis: A case report
BackgroundImmunoglobulin light chain amyloidosis is a clonal, non-proliferative plasma cell disorder, in which fragments of immunoglobulin light chain are deposited in tissues.
Yue Wang +5 more
doaj +1 more source
Bortezomib, Melphalan, and Dexamethasone for Light-Chain Amyloidosis [PDF]
PURPOSE Oral melphalan and dexamethasone (MDex) were considered a standard of care in light-chain (AL) amyloidosis. In the past decade, bortezomib has been increasingly used in combination with alkylating agents and dexamethasone. We prospectively compared the efficacy and safety of MDex and MDex with the addition of bortezomib (BMDex).
Kastritis, Efstathios +27 more
openaire +11 more sources
Immunoglobulin light chain amyloidosis and the kidney [PDF]
Immunoglobulin light chain amyloidosis and the kidney. Amyloidosis (AL) is a common cause of nephrotic syndrome in nondiabetic, nonhypertensive adults. All adult patients with nephrotic syndrome should have immunofixation of serum and urine as a screen.
Gertz, Morie A. +2 more
openaire +2 more sources
Localized Lymph Node Light Chain Amyloidosis
Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and ...
Binod Dhakal +3 more
doaj +1 more source

