Lipogranulomatous adenopathy: a characteristic but under-recognized presentation of Whipple's disease

Abstract

A50-year-old Caucasian male was admitted with a left popliteal vein thrombosis confirmed on Doppler ultrasound scanning. He was previously well and there was no obvious precipitating cause for his venous thrombosis. Apart from pallor and the swollen left calf there were no abnormal findings on routine examination and he was anticoagulated with heparin followed by warfarin. Investigations revealed a hypochromic microcytic anaemia (haemoglobin 8.3 g) and a marginally raised serum alkaline phosphatase. Other investigations including a myeloma screen, autoimmune profile and neutrophil cytoplasmic antibodies were negative. An intra-abdominal malignancy was suspected although faecal occult bloods were negative on three occasions and tumour markers (carcinoembryonic antigen (CEA) and CA 19.9) were normal. Upper gastrointestinal endoscopy demonstrated mild gastritis and a normal-looking duodenum only. Computed tomographic (CT) scanning of the abdomen and thorax revealed massive retroperitoneal and retrocrural adenopathy extending down to the small intestinal mesentery (Figure 1). The abdominal viscera were otherwise normal and there was no mediastinal Iymphadenopathy. Small bowel Iymphoma was suspected and he underwent first a laparoscopic biopsy of the mesenteric Iymph nodes and subsequently a laparotomy. There were no abnormal findings apart from the retroperitoneal Iymphadenopathy. On both occasions the histological appearances of the Iymph nodes were the same: the Iymph node architecture was destroyed by large lipid-containing spaces. There was also infiltration by numerous lipid containing macrophages, and occasional multinucleate giant cells (Figure 2). No cause for these changes could be recognized clinically or pathologically. Three months after his initial presentation he developed weight loss, mild diarrhoea, vague abdominal pain and a rash on his legs. A systemic vasculitis was suspected but a skin biopsy showed non-specific changes only. However, 1 week later he died suddenly at home, apparently having been markedly dyspnoeic for the preceding 48 hours. At postmortem he was found to have a severe constrictive pericarditis, which was felt to be the probable cause of his death. There was no evidence of pulmonary embolism. The liver showed congestive ‘nutmeg’ changes. There was evidence of a panserositis with inflammation of pericardium, pleura and peritoneum. Bowel histology was not performed because of autolysis. However, the histology of the mesenteric Iymph nodes was reviewed and on this occasion stained with periodic acid Schiff. This was taken up by bacilliary organisms in the macrophages found in the Iymph nodes. The features were considered diagnostic of Whipple's disease.