Patient-Reported Outcomes in Systemic Light-Chain Amyloidosis
نویسندگان
چکیده
منابع مشابه
Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders
Secondary amyloidosis can complicate chronic inflammatory autoimmune diseases. However, the clinical findings of primary amyloidosis may mimic those of primary rheumatologic disorders. We present the case of a 53-year-old woman who presented with dystrophic nail changes, dry eyes, bilateral carpal tunnel syndrome, Raynaud's phenomenon, and high titer positive nucleolar pattern antinuclear antib...
متن کاملHow we treat systemic light-chain amyloidosis.
Systemic light-chain (AL) amyloidosis is a multisystem disease characterized by organ toxicity and damage due to monoclonal free light chains, which are produced by a neoplastic clone of plasma cells in bone marrow. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains and thereby stop or reverse organ toxicity and damage. AL amyloidosi...
متن کاملHereditary systemic immunoglobulin light-chain amyloidosis.
Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing...
متن کاملSystemic light-chain amyloidosis: advances in diagnosis, prognosis, and therapy.
Systemic light-chain amyloidosis (AL) is caused by misfolded immunoglobulin light-chain proteins that aggregate and deposit as unique fibrils, ultimately leading to organ failure and death. Recent developments that have significantly aided the management of patients with AL include diagnostic techniques for definitive typing of amyloid deposits by use of laser microdissection with mass spectrom...
متن کاملCardiac magnetic resonance strain imaging in systemic light chain amyloidosis
Background Systemic light chain amyloidosis (AL) is associated with a high cardiovascular morbidity and mortality. Cardiovascular involvement and determination of prognosis is underestimated by standard imaging parameters. Recently, cardiac deformation analysis of global circumferential and longitudinal strain has been shown to have great clinical impact on the assessment of prognosis and survi...
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ژورنال
عنوان ژورنال: Blood
سال: 2018
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2018-99-111570