Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy characterized by dilatation of the lymphatics and loss lymph fluid into gastrointestinal tract, leading to development hypoproteinaemia, oedema, lymphocytopenia, hypogammaglobinaemia, immunologic abnormalities. We report series 4 children from Bangalore, India presenting with anasarca, diarrhoea, hypoproteinaemia. All four patients were confirmed have PIL on histopathology. retrospectively reviewed case records diagnosed primary department pediatric gastroenterology within time frame July 2015 September 2018. Four lymphangiectasia. them presented features protein losing enteropathy (generalized ascites low albumin), chronic lymphopenia, hypercalcemia, hypogammaglobulinemia. Endoscopically mucosa was normal in two showed Snowflake appearance other 2 patients. Intestinal biopsies characteristic all