Intestinal Lymphangiectasia: a Protein-Losing Enteropathy with Hypogammaglobulinemia, Lymphocytopenia and Impaired Homograft Rejection*

摘要: Intestinal lymphangiectasia is a disease characterized by dilated intestinal lymphatics, protein-losing enteropathy, hypoalbuminemia, and edema. The immunologic status of 18 patients with was studied. Concentrations IgG, IgA, IgM were measured immune precipitation metabolism these three immunoglobulins studied using purified radioiodinated proteins. serum concentration total body pool each immunoglobin greatly reduced. fraction the intravascular protein catabolized per day increased to 34% for 59% 66% IgM; are in contrast control values 7%, 28%, 17%, respectively. Synthetic rates normal or slightly increased. Primary circulating antibody response tested five Vi tularemia antigens. Titers elicited antigen significantly lower than those seen group, whereas no difference between patient responses antigen. Lymphocytopenia noted lymphangiectasia. mean lymphocyte count 710 +/- 340/mm(3) 2500 600/mm(3) controls. Cellular hypersensitivity skin tests grafts. 91% individuals reacted at least one four test antigens: derivative, mumps, Trichophyton, Candida albicans; contrast, only 17% had positive reaction. Each dinitrochlorobenzene negative Finally, all who received homografts have retained grafts 12 months. immunological disorders appear result from loss lymphocytes into gastrointestinal tract secondary lymphatic channels. Lymphocyte depletion then leads anergy impaired homograft rejection.