Xanthogranulomatous Cholecystitis in a Patient with Hemophilia A: A case report

Lim, Jin Chae; Roh, Jung Ho; Cho, Chol Kyoon; Kim, Hyun Jong

Abstract

Xanthogranulomatous cholecystitis (XGC) is an uncommon, focal or diffuse destructive inflammatory process of the gallbladder that is assumed to be a variant of chronic cholecystitis. XGC is characterized grossly by irregular thickening of the gallbladder wall with the formation of a yellow mass (xanthogranuloma). Histologically the xanthogranuloma appears as yellow nodules or streaks in a thickened gallbladder wall and is composed of predominantly lipid-laden macrophages, inflammatory cells, and fibroblasts. These xanthogranulomatous foci may extend into adjacent structures, adhesions or ulcerations are often present, and fistula formation may occur. Although the pathogenesis of XGC is unclear, recurrent inflammation in the presence of calculi and biliary stasis are thought to be the main etiological factors because of the histologic evidence of chronic inflammation and the presence of gallstones in a majority of cases. XGC may lead to associated complications such as perforation, abscess, and fistula, and sometimes closely mimics a gallbladder carcinoma. During an operation for XGC, careful surgical technique is required to dissect the gallbladder and to excise the adjacent xanthogranulomatous tissue. Also a careful investigation to find the coincident gallbladder carcinoma is necessary. We report a case with XGC in a hemophilia A patient and a review of the clinical literatures.