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Misdiagnosis of hereditary amyloidosis as AL (Primary) amyloidosis [PDF]
New England Journal of Medicine, 2002 Background: Hereditary, autosomal dominant amyloidosis, caused by mutations in the genes encoding transthyretin, fibrinogen A -chain, lysozyme, or apolipoprotein A-I, is thought to be extremely rare and is not routinely included in the differential ...
Booth, D.R. +7 more
core +8 more sources
Orphanet Journal of Rare Diseases, 2012 Abstract 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.
Éstelle Desport +13 more
+11 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
Etanercept AL amyloidosis [PDF]
The Lancet, 2001 Elizabeth Drewe, Richard J. Powell
openalex +3 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 +3 more sources
Be SMART About AL Amyloidosis [PDF]
Mayo Clinic Proceedings, 2021 n ...
Palladini, Giovanni, Merlini, Giampaolo
openaire +5 more sources
Blood, 2022 Abstract Light-chain amyloidosis has come far, with the first treatment getting regulatory approval in 2021. Daratumumab-based regimens achieve deep hematologic and organ responses, offering a new therapeutic backbone. Early identification, correct fibril typing, challenges of the very advanced patient, and lack of therapies to remove ...
Ashutosh D. Wechalekar +1 more
openaire +3 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.
openaire +7 more sources
Aggregation in AL Amyloidosis [PDF]
Biophysical Journal, 2014 Systemic light chain amyloidosis (AL amyloidosis) is a rare but fatal systemic amyloid disease. It usually strikes in the wake of myeloma, a form of cancer that affects plasma cells in the adaptive immune system. During plasma cell development from primary B cells, the immunoglobulin light chain (LC) genes undergo several rearrangements that leave each
Kathrin Andrich, Jan Bieschke
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Health-related quality of life in hereditary transthyretin amyloidosis polyneuropathy: a prospective, observational study [PDF]
, 2020 Background Hereditary Transthyretin Amyloidosis Polyneuropathy is a rare life-threatening neurologic disease that imposes considerable mortality and it is associated with progressive related disabilities.
Coelho, Teresa +5 more
core +2 more sources