Development of impaired splenic function in intestinal lymphangiectasia.

Abstract

SUMMARY We describe a patient with intestinal lymphangiectasia who developed hyposplenism and speculate that it resulted from chronic loss of lymphocytes into the gut.Splenic atrophy has been long recognised in association with coeliac disease' and, more recently, impaired splenic function has been described in patients with ulcerative colitis and Crohn's disease.2The aetiology of this hyposplenism is unknown, but by analogy with animal models it has been suggested that it results from lymphocyte depletion due to chronic loss into the gut.Intestinal lymphangiectasia is a disease characterised by dilated lymphatics, protein-losing enteropathy, hypoalbuminaemia and oedema, and patients with this disease lose albumin, immunoglobulins, and lymphocytes into the bowel. If the development of splenic atrophy results from prolonged lymphocyte depletion, it is likely that patients with intestinal lymphangiectasia will develop abnormalities of splenic function over a period of time, although, to our knowledge, this has not been studied.Since 1976, various tests of splenic function have been applied to one of our patients with intestinal lymphangiectasia and the results are reported below. During this time, however, the patient also developed other clinical problems.Case report A 40 year-old man presented in 1975 with a past history of vagotomy and pyloroplasty for a duodenal ulcer. He now complained of epigastric discomfort, diarrhoea, and ankle swelling. Examination confirmed pitting oedema of the ankles but there were

Infantile Intestinal Lymphangiectasia and Chylous Ascites in Saudi Arabia

“…Another rare finding in patients with IL is impaired splenic function [10], which was confirmed in two of our patients. IL usually affects the small intestine but it may affect both the small and large intestine [11].…”

Le syndrome d’APECED et la lymphangiectasie intestinale: une association fortuite ?

“…L'association décrite dans notre observation ne semble pas si fortuite. En effet, plusieurs similitudes sont observées entre le syndrome d'APECED et la lymphangiectasie intestinale primitive : l'anergie, l'hypoplasie thymique [14,15], l'atrophie splénique qui doit être systématiquement recherchée chez ces malades par une échographie abdominale [16,17], le défaut de l'émail dentaire et la dystrophie des ongles, ce qui suggère la possibilité d'une même étiologie et/ou d'un même mécanisme pathogénique. Ces données restent approximatives et non concluantes, en raison du caractère exceptionnel de cette association et surtout en raison de l'absence d'études affirmant ou infirmant le caractère fortuit ou non de cette association.…”

Hyposplenism and Gastrointestinal Diseases: Significance and Mechanisms

“…Case reports have demonstrated features of hyposplenism in various malabsorptive and inflammatory bowel disorders including tropical sprue, Whipple's disease, eosinophilic gastroenterological disorders, collagenous colitis, and intestinal lymphangiectasia [46][47][48][49][50] and in various hepatic disorders [7]. The extent and significance of hyposplenism in these bowel disorders have not been extensively studied.…”