title: Severe Refractory Anemia in Primary Intestinal Lymphangiectasia. A Case Report.
abstract: BACKGROUND
  Primary intestinal lymphangiectasia (Waldmann's disease) is a rare disease characterized by dilated lymphatics in the small bowel leading to an exudative enteropathy with lymphopenia, hypoalbuminemia and hypogammaglobulinemia.
  
  
  METHODS
  We report the case of a 23 year-old male who presented with chronic anemia and in whom primary intestinal lymphangiectasia was diagnosed. A low-fat diet along with nutritional therapy with medium-chain triglyceride supplementation improved the protein-losing enteropathy, but did not solve the anemia. Octreotide was also unsuccessful, and after attempting angiographic embolization therapy, limited small bowel resection together with antiplasmin therapy managed to correct the anemia and control the exudative enteropathy.
  
  
  CONCLUSIONS
  Although primary intestinal lymphangiectasia is usually adequately managed by nutritional therapy, complications such as anemia can occur and can prove to be a therapeutic challenge.
authors.affiliation: Dr. Carol Davila Central Military Emergency University Hospital; Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Vasile Daniel
authors.initials: VD
authors.lastname: Balaban
authors.affiliation: Carol Davila University of Medicine and Pharmacy; Alfred Rusescu Institute for Mother and Child Care, Bucharest, Romania; Tampere Center for Child Health Research, University of Tampere and Tampere University Hospital, Finland. alina.popp@uta.fi.
authors.forename: Alina
authors.initials: A
authors.lastname: Popp
authors.affiliation: Fundeni Clinical Institute, Bucharest, Romania.
authors.forename: Mugur
authors.initials: M
authors.lastname: Grasu
authors.affiliation: Dr. Carol Davila Central Military Emergency University Hospital, Bucharest, Romania.
authors.forename: Florina
authors.initials: F
authors.lastname: Vasilescu
authors.affiliation: Dr. Carol Davila Central Military Emergency University Hospital; Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
authors.forename: Mariana
authors.initials: M
authors.lastname: Jinga
mesh_terms: D000740:Anemia / Q000097:blood / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D001706:Biopsy; D002908:Chronic Disease; D018752:Diet, Fat-Restricted; D019587:Dietary Supplements; D013505:Digestive System Surgical Procedures; D004621:Embolization, Therapeutic; D016099:Endoscopy, Gastrointestinal; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008209:Lymphedema / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D008297:Male; D015282:Octreotide / Q000627:therapeutic use; D011504:Protein-Losing Enteropathies / Q000209:etiology / Q000628:therapy; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014280:Triglycerides / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D000933:Antifibrinolytic Agents; D014280:Triglycerides; D015282:Octreotide