심장을 침범한 Systemic Amyloidosis 1예
A Case of Systemic Amyloidosis
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초록
저자들은 58세 여자에게서 심장판막 역류의 정도가 심한 변화를 보이며 신장 기능 이상과 간, 비대, 심비대를 동반한 전신적 아밀로이드증 1예를 경험하였기에 이에 문헌과 함께 보고하는 바이다.
Abstract
Systemic amyloidosis is an uncommon disease characterized by deposits of fibrillar aggregates of monoclonal immunoglobuloin light chains in vital organs. This amyloid deposit cause cardiac or renal dysfunction and ultimately, death. Cardiac amyloidosis may be asymptomatic or important causes of progressive heart failure and refractory arrhythmia. Cardiac involvement from AL amyloidosis is rapidly fatal. The amyloidoses are classified according to the biochemical nature of the fibril-forming protein. Cardiac amyloidosis is common in primary (AL) and heterofamilial amyloidosis and very rare in the secondary (AA) form. As we experienced a case of systemic amyloidosis affected heart, liver and kidney, which was confirmed by histology. We present a 57-year-old female case with literature review.