oa South African Medical Journal - Primary systemic amyloidosis with gross cardiac involvement
Primary amyloidosis differs from secondary amyloidosis mainly in that there is no primary pathology to account for the condition, and the amyloid is deposited in the heart, lungs, skin and striated muscle rather than in liver, spleen and kidney. A case is presented of primary systemic amyloidosis characterized clinically by congestive cardiac failure of unknown etiology not responding to intensive therapy. At autopsy, the heart was grossly enlarged due to amyloid deposition in the myocardium. Myloid was also found in the small arteries and arterioles, alveolar walls of the lung, muscle of the gastric wall and uterus, and round the fat cells in the omentum and subpericardial connective tissues. Primary amyloidosis should be considered more frequently in cardiac failure of unknown etiology in the older age groups especially where there is no response to adequate treatment.
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