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Primary Intestinal Lymphangiectasia

Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites and protein-losing enteropathy. We report a case of PIL in a 22 year old patient from Tabriz, Iran who presented with anasarca edema, recurrent diarrhea, hypoproteinemia and confirmatory features of PIL on endoscopy and histopathology. Therefore we advise the consideration of PIL in the differential diagnosis of chylous ascitis. Keywords: Hypogammaglobulinemia, nodular lymphoid hyperplasia, primary intestinal lymphangiectasia (PIL), chylous ascitis