title: Primary intestinal and thoracic lymphangiectasia: a response to antiplasmin therapy.
abstract: Lymphangiectasia is a congenital or acquired disorder characterized by abnormal, dilated lymphatics with a variable age of presentation. We describe a case of lymphangiectasia with intestinal and pulmonary involvement in an adolescent female, who presented with many of the classic features including chylous pleural effusions, lymphopenia, hypogammaglobinemia, and a protein-losing enteropathy. She also presented with recurrent lower gastrointestinal bleeding, which is infrequently described. The patient did not improve with bowel rest and a low-fat medium-chain triglyceride diet and had little improvement with octreotide acetate therapy. However, she had a clinical response to antiplasmin therapy, trans-4-aminothylcyclohexamine carboxylic acid (tranexamic acid) in terms of serum albumin and gastrointestinal bleeding. She continues to have exacerbations of her condition, as well as persistent lymphopenia and chronic pleural effusions.
authors.affiliation: Division of Paediatric Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada M5G 1X8.
authors.forename: Joanna E
authors.initials: JE
authors.lastname: MacLean
authors.forename: Eyal
authors.initials: E
authors.lastname: Cohen
authors.forename: Michael
authors.initials: M
authors.lastname: Weinstein
mesh_terms: D000293:Adolescent; D000933:Antifibrinolytic Agents / Q000627:therapeutic use*; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000188:drug therapy; D006801:Humans; D008200:Lymphangiectasis / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy*; D008201:Lymphangiectasis, Intestinal / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy*; D013902:Radiography, Thoracic; D012709:Serum Albumin / Q000032:analysis; D013896:Thoracic Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy*; D014148:Tranexamic Acid / Q000627:therapeutic use; D016896:Treatment Outcome; D000979:alpha-2-Antiplasmin / Q000627:therapeutic use*
chemical_list: D000933:Antifibrinolytic Agents; D012709:Serum Albumin; D000979:alpha-2-Antiplasmin; D014148:Tranexamic Acid