Studies of the Immunologic Defects Associated with Intestinal Lymphangiectasia

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Abstract
Studies in a patient with lymphangiectasia, lymphedema, lymphocytopenia and hypogammaglobulinemia suggest that immunologic defects in such patients are correlated with excessive globulin catabolism rather than an intrinsic defect in immune competence. Fluorescein-labelling techniques showed that immunoglobulin-bearing cells of the lamina propria of the affected intestine were present in approximately normal number and distribution in spite of reduced levels of serum [gamma]G-,[gamma]A- and [gamma]M-immunoglobulins. The patient was capable of producing serum reagin antibodies which are probably YE immunoglobulins. Normal numbers of lymphocytes in blood and ascitic fluid underwent transformation in response to stimulation by phytohemag-glutinin and specific antigen. A unique feature of this patient with lymphangiectasia was the presence of cutaneous lymphangiomata. Long-term success in control of associated chylous ascites was achieved by a low fat diet.