Abdominal Lymphoma Presenting with Malabsorption

Abstract

Twenty-three cases of abdominal lymphoma presenting with malabsorption seen at Groote Schuur Hospital over a 10-year period are reviewed. The clinical features, haematology, biochemistry, absorptive functions, radiologic and histological features are presented. Diarrhoea was a feature in all the cases while steatorrhoea occurred in 21. Intermittent abdominal pain, finger clubbing, and peripheral oedema were present in 50 per cent of the cases. Hepatosplenomegaly and abdominal masses were not frequent findings. Haematological investigations were similar to those found in any malabsorption syndrome. The erythrocyte sedimentation rate was above normal in 15 cases, but only above 30 mm/h in 10 cases. Routine tests of malabsorption were abnormal in most cases. Barium studies of the small bowel were abnormal in all 19 cases in which they were performed, and in six cases the diagnosis of lymphoma was suggested by the radiologist. Lymphangiography was of value in the four cases in which this procedure was carried out. The diagnostic value and drawbacks of peroral jejunal biopsy are stressed, lymphoma being present in biopsies of five patients and in five patients biopsies were completely normal. Thus in this series this suggests that peroral jejunal biopsies will be diagnostic in one-third, suggestive in one-third, and normal in one-third of patients with malabsorption due to lymphoma. Attention is drawn to the findings of total or partial villous atrophy, combined with 'plasma cell transformation' in the lamina propria. The results are compared to other series. Primary lymphoma and lymphoma secondary to idiopathic steatorrhoea is discussed and reasons are advanced to show that the cases in the present series were not secondary to idiopathic steatorrhoea. The possible pathogenesis and relationship to immunoglobulin deficiencies is discussed. The role of environmental factors is noted. The difficulties in making a diagnosis are mentioned and the importance of lymphoma as a cause of malabsorption in the Western Cape is stressed. The use of the term ‘Mediterranean type’ of abdominal lymphoma is regarded as a misnomer.