Journal of the Korean Society of Echocardiography  2000;8(1):87-92

A Case of Systemic Amyloidosis.

Gyung Ho YOON 1 ; Chang Soo CHOI ; Suk Gyu OH ; Jin Won JUNG ; Yang Gyu PARK ; Ok Gyu PARK ; Gyung Hee KIM ; Woo Geun SONG

Affiliations

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Keywords

Systemic amyloidosis

Country

Republic of Korea

Language

Korean

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.