Histopathological study of multi-systemic AA amyloidosis with cardiac involvement: two fatal cases in forensic practice

Abstract

In this report, two cases of death in patients diagnosed with multi-systemic AA amyloidosis at autopsy were investigated for any medical errors that may have contributed to their deaths. The patients developed nephrotic syndrome post-operatively and following trauma. The second patient had been on hemodialysis for idiopathic kidney disease prior to the trauma she sustained. Complete autopsies with immunohistochemistry and electron microscopy were used to show the extracellular deposits of amyloid in the kidneys and also in other organs, including the heart. Cardiac involvement for this type of amyloidosis has never been reported in the literature. Post-mortem analysis showed multi-systemic AA amyloidosis as the cause of nephrotic syndrome, and ultimately the cause of worsening clinical outcomes, for the two patients. A comprehensive histopathological study led to a peculiar organ-specific observation of AA amyloidosis, which requires more thorough investigations by clinicians and must be considered in the evaluation by forensic pathologists in cases of professional liability.

Keywords:

• multi-systemic AA amyloidosis
• cardiac involvement
• histopathological study
• professional liability
• forensic approach