Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology · 2010
Behind heart failure syndrome: remember AL amyloidosis. Two case-reports.
Клинический случайСтатьяНазвание и аннотация: Перевод пока не подготовлен
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Silva Doroteia, Sargento Luís, Varela Manuel Gato, Brito Dulce, Lopes Mário G
Аннотация
Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins. Identification of the amyloidogenic protein determines the type of amyloidosis. In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia. Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients. The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death). The authors wish to draw attention to an entity that is rarely encountered in clinical practice and that requires a high index of suspicion.