Studies of the Immunologic Defects Associated with Intestinal Lymphangiectasia

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