Autopsy case of Dubin-Johnson syndrome with pneumonia and abetalipoproteinemia-like lipid profile.

We report the autopsy of a 79-year-old Japanese woman with Dubin-Johnson syndrome accompanied by pneumonia, an abetalipoproteinemia-like lipid profile and acanthocytosis. On admission, physical examination of the patient revealed malnutrition. Blood tests revealed marked inflammatory changes and mild liver dysfunction. Chest X-ray indicated bilateral pneumonia. Total cholesterol, low-density lipoprotein (LDL) cholesterol and triglyceride levels were 89 mg/dL, 5 mg/dL and 6 mg/dL, respectively. Peripheral blood smears revealed numerous acanthocytes. Despite the administration of antibiotics and nutritional support, the patient died. Autopsy revealed a black liver, atrophy of fat tissue on the mesentery, and pneumonia with bilateral pleural effusion. We believe that the abetalipoproteinemia-like lipid profiles in this case were caused by malnutrition and the inflammatory changes rather than the direct effects of Dubin-Johnson syndrome. We base this conclusion on the following three findings: 1) the patient's lipid profile before hospitalization was in the normal range, 2) her serum LDL cholesterol and triglyceride levels gradually increased after nutritional support began, and 3) blood tests revealed marked inflammatory changes (C-reactive protein 9.0 mg/dL; interleukin-6 16.4 pg/mL). This case provides important information that enhances our understanding of lipid metabolism under conditions of malnutrition and inflammation.