A Case of Primary Intestinal Lymphangiectasia Presented With Small Bowel Bleeding and Improved by Antiplasmin Therapy: Presidential Poster

Abstract

Primary intestinal lymphangiectasia (PIL) is a rare disorder of intestinal lymphatic channel malformation and is characterized by dilated lacteals and impaired lymphatic drainage leading to leakage of lymph into intestinal lumen resulting in lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The diagnosis is based on histology and exclusion of secondary causes such as congestive heart failure, nephrotic syndrome, inflammatory bowel disease, rheumatologic disease, infectious disease, etc. A low fat, high protein, medium-chain triglyceride supplemented diet is mainstay of management. Octreotide and tranexamic acid is reported to decrease lymphatic permeability and can be used for laboratory and symptomatic improvement. We report a case of PIL which presented with small bowel bleeding and improved in clinical and laboratory abnormalities with antiplasmin therapy. A 32 year-old female visited emergency department for melena and peripheral edema. Laboratory finding revealed anemia, hypoalbuminemia and lymphopenia. Gastroduodenoscopy showed that whitish spots and finger like elongated mucosal lesion was scattered in the second portion of the duodenum. Colonoscopy revealed that tarry stool was shown and nodular lesion with whitish spots was scattered at terminal ileum, but colon mucosa was intact. Capsule endoscopy revealed that bleeding was shown at proximal jejunum and small bowel villi was elongated with whitish spots and chylous products gushed out. Small bowel biopsy showed dilated lymphatics in mucosa and submucosa. Secondary causes of lymphatic obstruction like heart failure, liver cirrhosis, retroperitoneal fibrosis, mesenteric venous thrombosis, neoplasm excluded. And also, mucosal diseases increased lymphatic permeability such as inflammatory bowel disease, rheumatic disease, allergic disease, gastrointestinal malignancy, infection, celiac disease, amyloidosis ruled out. Bleeding and edema continued despite of medium-chain triglycerides supplementation. As antiplasmin therapy was administered, melena and edema decreased and lymphopenia and hypoalbuminema improved. We reports a case of primary intestinal lymphangiectasia presented with small bowel bleeding, and improved clinical and laboratory findings by MCT diet and antiplasmin therapy.Table 1: Comparison of laboratory test before and after dischargeFigure 1Figure 2Figure 3