Systemic light-chain amyloidosis presenting with rapidly progressive heart failure
نویسندگان
چکیده
منابع مشابه
Senile systemic amyloidosis presenting with heart failure: a comparison with light chain-associated amyloidosis.
BACKGROUND Small deposits of amyloid are often found in the hearts of elderly patients. However, extensive deposition of transthyretin-derived amyloid fibrils in the heart (senile systemic amyloidosis [SSA]) can cause heart failure. The clinical features of SSA that involve the heart are ill defined, and the condition may be overlooked as a cause of heart failure. We sought to better define the...
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BACKGROUND Light-chain amyloidosis (AL) with cardiac involvement carries a poor prognosis; median untreated survival is <6 months. Three-drug therapy with bortezomib, dexamethasone, and an alkylating agent (BDex+AA) is associated with improved biomarker response rates in AL amyloidosis. OBJECTIVES This study sought to evaluate the effect of BDex+AA as a first-line treatment strategy on mortal...
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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...
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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...
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ژورنال
عنوان ژورنال: European Heart Journal
سال: 2013
ISSN: 0195-668X,1522-9645
DOI: 10.1093/eurheartj/eht312.4520