Tohoku J. Exp. Med., 2022 December, 258(4)

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

Tetsuya Horita,¹ Naoki Kosaka,² Shinya Takaoka,³ Gento Fujii,⁴ Kana Fujimoto,¹ Yoshihito Koshimizu,¹ Tsuneo Kakuda,¹ Hideki Shojo¹ and Noboru Adachi¹

¹Department of Legal Medicine, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Chuo, Yamanashi, Japan
²Faculty of Medicine, University of Yamanashi, Chuo, Yamanashi, Japan
³Department of Gastroenterology and Hepatology, University of Yamanashi Hospital, Chuo, Yamanashi, Japan
⁴Laboratory of Systems Genomics, Department of Computational Biology and Medical Sciences, Graduate School of Frontier Sciences, The University of Tokyo, Kashiwa, Chiba, Japan

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 —— autopsy; hyposplenism; systemic lupus erythematosus; vaccine; Waterhouse-Friderichsen syndrome

© 2022 Tohoku University Medical Press

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Tohoku J. Exp. Med 2022, 258, 287-301.

Correspondence: Tetsuya Horita, Department of Legal Medicine, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, 1110 Shimokato, Chuo, Yamanashi 409-3898, Japan.

e-mail: htetsuya@yamanashi.ac.jp