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Orphanet Journal of Rare Diseases, 2012 Definition of the disease AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone.
Desport Estelle +13 more
doaj +11 more sources
Management of AL amyloidosis in 2020 [PDF]
Hematology, 2020 Abstract In amyloid light chain (AL) amyloidosis, a small B-cell clone, most commonly a plasma cell clone, produces monoclonal light chains that exert organ toxicity and deposit in tissue in the form of amyloid fibrils. Organ involvement determines the clinical manifestations, but symptoms are usually recognized late.
Palladini, G., Milani, P., Merlini, G.
semanticscholar +9 more sources
Biomarkers in AL Amyloidosis [PDF]
International Journal of Molecular Sciences, 2021 Systemic AL amyloidosis is a rare complex hematological disorder caused by clonal plasma cells which produce amyloidogenic immunoglobulins. Outcome and prognosis is the combinatory result of the extent and pattern of organ involvement secondary to amyloid fibril deposition and the biology and burden of the underlying plasma cell clone.
Despina Fotiou +2 more
openaire +4 more sources
Misdiagnosis of Hereditary Amyloidosis as AL (Primary) Amyloidosis [PDF]
New England Journal of Medicine, 2002 Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A alpha-chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential diagnosis of systemic amyloidosis unless there is a family history.We studied 350 patients with systemic ...
Helen J. Lachmann +7 more
openalex +6 more sources
A RARE PRESENTATION OF SYSTEMIC AL AMYLOIDOSIS; PULMONARY AL AMYLOIDOSIS
Hematology, Transfusion and Cell Therapy, 2022 Objective: Involvement of the lung is common in systemic AL amyloidosis in post-mortem series. However, the diagnosis is challenging. Histology is the gold standard but may result in bleeding. Consequently, diagnosis during life is rare.
Simge ERDEM +4 more
doaj +2 more sources
Perspectives in treatment of AL amyloidosis [PDF]
British Journal of Haematology, 2007 SummaryLight chain (AL) amyloidosis is the most frequently diagnosed form of systemic amyloid in the western world. The historically poor prognosis of AL amyloidosis appears to be improving with currently reported median survival of c. 40 months compared to 13 months in the early 1990s when low‐dose oral melphalan was the mainstay of treatment ...
Ashutosh D. Wechalekar +2 more
openalex +4 more sources
Epidemiology of AL amyloidosis: a real-world study using US claims data
Blood Advances, 2018 : Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States ...
Tiffany P. Quock +4 more
doaj +2 more sources
Nature Communications, 2019 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 +2 more sources
The Lancet, 2013 A 62-year-old man came to our outpatient clinic with purpura on his eyelids. 7 months before, periocular purpura appeared after a minimal trauma. These lesions did not resolve for months. He also reported fatigue over the previous 6 months. On examination pinch purpura between smooth waxy papules were found (fi gure).
Barbara C. Böckle, Norbert Sepp
openalex +3 more sources
Etanercept AL amyloidosis [PDF]
The Lancet, 2001 Elizabeth Drewe, Richard J. Powell
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