Immunological Study in Primary Intestinal Lymphangiectasia

Abstract

Primary intestinal lymphangiectasia is a rare congenital condition associated with protein-losing enteropathy. Hypogammaglobulinemia and lymphopenia secondary to this condition are frequent but infectious complications are not. So far few immunological studies have been made in these patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease in serum gammaglobulins, especially IgG and IgA, and in peripheral blood lymphocytes, especially CD4 T helper cells. From a functional standpoint, the proliferative response to certain mitogens was reduced. A decrease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of duodenal biopsy specimens revealed a decreased number of intraepithelial lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in the terminal ileum, confirmed by endoscopic biopsy. The role of these abnormalities in the development of infectious complications and lymphoma is underscored.

Circumscribed intestinal protein loss with deficiency in CD4+ lymphocytes after the Fontan procedure

“…Moreover, all these patients showed further immunological abnormalities involving de®ciency of immunoglobins of all isotypes [6,18], hypoplasia of the thymus [16], impairment of in vitro Bcell function [18], disturbance of in vitro T-cell function [6], and increased suppressor activity [18]. Bacterial infections were more common [6,16]. In contrast, our patient did not acquire opportunistic or severe infections.…”

“…The CD4+/CD8+ ratio in peripheral blood lymphocytes, however, was 1.45 [10]. Only in some patients with primary intestinal lymphangiectasia has an inversion of the CD4+/CD8+ ratio been observed [6,16,18]. These patients, however, developed severe diarrhoea [6,16,18] and an excessive intestinal protein loss [18].…”

“…Only in some patients with primary intestinal lymphangiectasia has an inversion of the CD4+/CD8+ ratio been observed [6,16,18]. These patients, however, developed severe diarrhoea [6,16,18] and an excessive intestinal protein loss [18]. Moreover, all these patients showed further immunological abnormalities involving de®ciency of immunoglobins of all isotypes [6,18], hypoplasia of the thymus [16], impairment of in vitro Bcell function [18], disturbance of in vitro T-cell function [6], and increased suppressor activity [18].…”

Classification of Intestinal Lymphangiectasia with Protein-Losing Enteropathy: White Villi Type and Non-White Villi Type

“…This gastric non-cirrhotic snakeskin appearance could be a clue in diagnosing the non-white villi type. Conversely, lymphocytes and intestinal lymph containing immunoglobulin drains directly into the intestinal lumen through ruptured or permeable lymphatics in the lamina propria; therefore, the white villi type might preferentially have resulted in severe lymphocytopenia and hypoimmunoglobulinemia [16]. …”

Digital clubbing in primary intestinal lymphangiectasia: a case report

“…It has been consistently noted even during a course of immunoglobulin substitution. Patients with PIL have immunological abnormalities involving both the B-cell and T-cell lineages of the immune system; in combination with a secondary immune deficiency resulting from the loss of immunoglobulins and lymphocytes, a B-cell defect in PIL was characterized by low Ig levels (IgG, IgA and IgM) and poor antibody responses [18]. Since in our patient, low immunoglobulin levels were associated with the presence of a monoclonal gammopathy IgM kappa, hematological follow-up was initiated.…”