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Be SMART About AL Amyloidosis [PDF]
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Palladini, Giovanni, Merlini, Giampaolo
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Background Systemic amyloidosis is caused by the deposition of misfolded protein aggregates in tissues, leading to progressive organ dysfunction and death.
Roberta Shcolnik Szor+15 more
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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
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BackgroundWhile patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging.
Zsofia Dohy+11 more
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Systemic Immunoglobulin Light Chain Amyloidosis Presenting as Painful Penile Ulcers
Immunoglobulin light chain (AL) amyloidosis is a systemic form of amyloidosis that commonly affects the heart, kidney, liver, and nervous system. Penile involvement in AL amyloidosis is rare and thus often not considered in the differential diagnosis for
Crystal Lihong Yan+3 more
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Management of AL amyloidosis in 2020 [PDF]
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.
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Aggregation in AL Amyloidosis [PDF]
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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Supportive Care in AL Amyloidosis [PDF]
Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response.
Joan Bladé+5 more
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Difficulties in diagnosis of primary AL-amyloidosis
Objective of the study: analysis of AL-amyloidosis (AL-A) diagnostics in real clinical practice and to determine the main approaches for the earlier detection of this disease.Materials and methods.
V. A. Khyshova+3 more
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Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report [PDF]
Gastrointestinal amyloidosis is a condition caused by the deposition of extracellular protein fragments. It can be associated with complex and diverse pathways and can have numerous manifestations and etiologies.
Zahra Davoudi+3 more
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