Three Autopsy Cases of Non-Meningococcal Waterhouse-Friderichsen Syndrome with Hypoplastic Spleen or Post-Splenectomy Status

Tohoku J Exp Med. 2022 Nov 12;258(4):287-301. doi: 10.1620/tjem.2022.J085. Epub 2022 Oct 20.

Abstract

We report three cases of Waterhouse-Friderichsen syndrome (WFS) that were confirmed during forensic autopsies. Case 1 involved a man in his 50s post-splenectomy. Bacteriological examination revealed Streptococcus pneumoniae (S. pneumonia). The patient was considered to have died of asphyxiation after aspirating vomit. Case 2 involved a man in his 40s. Bacteriological examination again revealed S. pneumoniae. Histopathological examination showed hypoplasia of the spleen. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. Case 3 involved a post-splenectomy woman in her 60s with a history of systemic lupus erythematosus. Bacteriological examination revealed Streptococcus oralis. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. These three cases were included among forensic autopsies conducted in the last 5 years. WFS has been considered a rare disease, but may be more frequent than previously assumed. If a mildly ill patient displays a sudden change in status and dies within a short period of time, we consider it necessary to perform not only bacteriological examinations, but also histopathological examination of the spleen during autopsy.

Keywords: Waterhouse-Friderichsen syndrome; autopsy; hyposplenism; systemic lupus erythematosus; vaccine.

MeSH terms

  • Autopsy
  • Disseminated Intravascular Coagulation* / etiology
  • Female
  • Humans
  • Male
  • Sepsis*
  • Spleen / pathology
  • Splenectomy
  • Waterhouse-Friderichsen Syndrome* / diagnosis
  • Waterhouse-Friderichsen Syndrome* / pathology