LEUKOCYTE ADHESION DEFICIENCY PRESENTING AS A CHRONIC ILEOCOLITIS

CD11/CD18 leucocyte glycoprotein deficiency is a rare, congenital adhe sion molecule disorder which, in its severe form, is usually fatal. Le ucocytes in affected subjects have abnormal migration and adherence, r endering patients susceptible to life threatening infections. The CD11 /CD18 integrins, and other adehsion molecules, are considered essentia l to the normal inflammatory response. It has been postulated that adh esion molecules may be responsible for mediating in part, the inflamma tory changes observed in inflammatory bowel diseases and related disor ders. This report describes the first case of CD11/CD18 deficiency cha racterised by a chronic ileocolitis. Bone marrow transplantation compl etely resolved the gastrointestinal symptoms, supporting a role for ne utrophil dysfunction in the pathogenesis of the gut lesions. This case suggests that specific blockade of CD11/CD18 integrins alone may not halt the chronic inflammatory response observed in immune mediated bow el disorders, and that abnormalities of leucocyte function must be inc luded in the differential diagnosis of paediatric Crohn's disease.