Rapidly progressive AA cardiomyopathy

W. Ishii, B. Kluve-Beckerman, J. J. Liepnieks, S. T. Vakili, M. D. Benson

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Abstract

Cardiac involvement may occur with AA amyloidosis, but extensive deposition in the heart is rarely seen. We report a 58-year-old man who on autopsy showed massive cardiac AA amyloid deposition. He did not have history of a chronic inflammatory condition and died suddenly prior to diagnosis of amyloidosis. Autopsy revealed marked left ventricular hypertrophy. Although extensive eosinophilic deposits were present in cardiac and adrenal tissues, the deposits stained weakly with Congo red. Biochemical analysis of fibrils extracted from heart gave a protein sequence showing identity to serum amyloid A. Immunohistochemistry with anti-human AA monoclonal antibody revealed strong staining of amyloid deposits in cardiac and adrenal tissues. Based on these findings, a diagnosis of systemic AA amyloidosis was made. This case highlights the importance of considering AA amyloidosis when the causes of left ventricular hypertrophy are unknown, and demonstrates the usefulness of biochemical analysis for diagnosing amyloid type.

Original languageEnglish (US)
Pages (from-to)203-205
Number of pages3
JournalAmyloid
Volume18
Issue numberSUPPL. 1
DOIs
StatePublished - Jun 1 2011

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ASJC Scopus subject areas

  • Internal Medicine

Cite this

Ishii, W., Kluve-Beckerman, B., Liepnieks, J. J., Vakili, S. T., & Benson, M. D. (2011). Rapidly progressive AA cardiomyopathy. Amyloid, 18(SUPPL. 1), 203-205. https://doi.org/10.3109/13506129.2011.574354