Korean J Leg Med.  2023 Feb;47(1):14-20. 10.7580/kjlm.2023.47.1.14.

Cardiac Amyloidosis in Autopsy Case of Sudden Unexpected Death

Affiliations
  • 1Department of Pathology, Pusan National University Yangsan Hospital, Yangsan, Korea
  • 2Department of Forensic Medicine, Pusan National University School of Medicine, Yangsan, Korea
  • 3Forensic Medicine Division, National Forensic Service Busan Institute, Yangsan, Korea
  • 4Hyundai Forensic Medical Service, Seoul, Korea

Abstract

A 78-year-old woman with a medical history of hypertension and diabetes mellitus who underwent surgery for lumbar stenosis died of sudden cardiac arrest two days after the operation. An autopsy was performed; however, the cause of death was not identified macroscopically. Congo red staining detected amyloid deposits in the systemic organs, including the heart, lungs, liver, thyroid, and kidney. Immunohistochemical staining revealed an immunoglobulin lambda light chain, which can cause the primary form of systemic amyloidosis. The prognosis of patients with systemic amyloidosis is directly associated with cardiac involvement. In this case, amyloid formation was noted in the myocardial interstitium and intramyocardial vascular wall, which caused luminal narrowing, subsequently causing arrhythmia and ischemic heart disease in each tissue, respectively. We present a case of primary systemic amyloidosis with severe cardiac involvement that was diagnosed after a comprehensive postmortem examination.

Keyword

AL amyloidosis; Autopsy; Forensic pathology; Heart; Sudden cardiac death
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