IMMUNOLOGICAL STUDY IN PRIMARY INTESTINAL LYMPHANGIECTASIA

Primary intestinal lymphangiectasia is a rare congenital condition ass ociated with protein-losing enteropathy. Hypogammaglobulinemia and lym phopenia secondary to this condition are frequent but infectious compl ications are not. So far few immunological studies have been made in t hese patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease i n serum gammaglobulins, especially IgG and IgA, and in peripheral bloo d lymphocytes, especially CD4 T helper cells. From a functional standp oint, the proliferative response to certain mitogens was reduced. A de crease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of d uodenal biopsy specimens revealed a decreased number of intraepithelia l lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in th e terminal ileum, confirmed by endoscopic biopsy. The role of these ab normalities in the development of infectious complications and lymphom a is underscored.