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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
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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
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Chronic Viral Infections and Al Amyloidosis: An Uncommon Association [PDF]
Clinical Case ReportsAL‐amyloidosis is caused by deposition of pathological insoluble proteins in organs and tissues. HIV infection is a multi‐system disorder causing chronic inflammation.
Alberto Palladini +7 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 Wechalekar +2 more
openalex +4 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
Etanercept AL amyloidosis [PDF]
The Lancet, 2001 Elizabeth Drewe, Richard J. Powell
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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 +3 more sources
Timing and co-occurrence of symptoms prior to a diagnosis of light chain (AL) amyloidosis [PDF]
Blood Cancer JournalIt is well-established that most patients with systemic light chain (AL) amyloidosis have multi-organ involvement and are often diagnosed after a lag period of increasing symptoms. We leverage electronic health record (EHR) data from the TriNetX research
Ashima Singh +5 more
doaj +2 more sources
IgM-Related Immunoglobulin Light Chain (AL) Amyloidosis
Hemato, 2022 Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic disorder characterized by an IgM paraprotein. The clinical presentation of WM varies and can include common manifestations such as anemia and hyperviscosity, in addition to less common ...
Shayna Sarosiek +3 more
doaj +1 more source
Diagnosis for Chinese patients with light chain amyloidosis: a scoping review
Annals of Medicine, 2023 Background Amyloid light chain (AL) amyloidosis is the most common systemic amyloidosis. The objective of this scoping review was to map the available literature on the diagnosis of AL amyloidosis in China.Materials and Methods The published academic ...
Meilan Chen +9 more
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