A case of hereditary amyloidosis transthyretin variant Met 30 with amyloidcardiomyopathy, less polyneuropathy, and the presence of giant cells

Citation
Y. Nakamura et al., A case of hereditary amyloidosis transthyretin variant Met 30 with amyloidcardiomyopathy, less polyneuropathy, and the presence of giant cells, PATHOL INT, 49(10), 1999, pp. 898-902
Citations number
16
Categorie Soggetti
Medical Research Diagnosis & Treatment
Journal title
PATHOLOGY INTERNATIONAL
ISSN journal
13205463 → ACNP
Volume
49
Issue
10
Year of publication
1999
Pages
898 - 902
Database
ISI
SICI code
1320-5463(199910)49:10<898:ACOHAT>2.0.ZU;2-8
Abstract
Transthyretin-Met 30 (TTR-Met 30) is a variant of transthyretin and is usua lly associated with familial amyloid polyneuropathy. It is rare that patien ts with TTR-Met 30 will primarily develop amyloid cardiomyopathy. This repo rt presents a patient with late-onset TTR-Met 30 who primarily developed am yloid cardiomyopathy, with less amyloid polyneuropathy in the peripheral ne rvous system than is usually seen. An autopsy was performed, and histologic al examination revealed many foreign-body giant cells and macrophages in th e area of amyloid deposition that was found in nearly all of the organs.