Hepatic and appendiceal Whipple's disease with negative jejunal biopsies

A 20-year-old man with Whipple's disease, right upper quadrant pain, abnormal liver tests and multiple negative peroral small bowel biopsies is presented. The patient underwent laparotomy: foamy macrophages were noted in the appendix and mesenteric lymph nodes; using electron microscopy, bacillary bodies were seen in the appendix, the mesenteric lymph node, and the liver. This patient had the rare occurrence of Whipple's disease of the appendix with bacillary bodies in the liver and a clinical picture compatible with hepatic involvement or cholangitis and a slow response to conventional antibiotic therapy.