IMMUNODEFICIENCY DUE TO CHYLOUS DYSPLASIA: DIAGNOSTIC AND THERAPEUTIC CONSIDERATIONS

Among primary immunodeficiencies,common variable immunodeficiency (CVID)is defined by an impaired production ofimmunoglobulins characterized by low levelsof plasma immunoglobulins and an alteredantibody response. The case reported here wasinitially interpreted as a CVID. A 20 year oldmale suffered from diarrhea, weight loss, andmalnutrition. Accurate diagnostic assessmentuncovered a protein-losing enteropathy.Conventional oil contrast lymphangiographyaccurately documented the underlyingproblem and established the appropriatetherapeutic approach. The operation consistedof multiple antigravitational ligatures ofdilated and incompetent chylous vessels andchylous vessel-mesenteric vein microanastomoses.Serum albumin and leukocyte countsnormalized by 1 week after operation andremained stable with time. There were nomore episodes of diarrhea, and the patientregained weight. Accurate diagnosticassessment and particularly lymphangiographymay be necessary to properly define difficultcases of immunodeficiency due to intestinalprotein loss and to plan a correctivetherapeutic functional approach.