Z Gastroenterol 2004; 42 - 37
DOI: 10.1055/s-2004-826938

Refractory sprue: Report of 2 cases without previous history of celiac disease

T Gurbity Palfi 2, L Krenacs 1, E Bagdi 1, T Krenacs 1, K Piukovics 2, J Kiss 3
  • 1Laboratory of Tumour Pathology and Molecular Diagnostics, Institutes of Biotechnology, Bay Zoltan Fo
  • 22nd Department of Internal Medicine, University of Szeged
  • 3Department of Internal Medicine, City Hospital, Szeged

Background: Refractory sprue (RS) fails to improve after a strict gluten-free diet. Intraepithelial lymphocytes (IEL) in RS show abnormal phenotype. Our report presents 2 cases of RS with no previous history of celiac disease (CD).

Case reports: Case 1: 71 years old female was diagnosed and treated for anaplastic large cell lymphoma presented in an axillary lymph node. Gastroscopy biopsy performed due to profuse diarrhea revealed flat mucosa (Marsch 3c) with marked theliocytosis (10 IEL/10 epithelial cells). IEL demonstrated CD3+, CD8-, strongly suggestive of RS. PCR revealed monoclonal T-cell gene rearrangement. No previous history of CD was known. Patient died due to severe malnutrition and cachexia irrespective of strict gluten-free diet. Case 2: 65 years old female was admitted to hospital due to anorexia. Gastroscopy showed granulation and multiple ulcers in duodenal mucosa. Biopsy demonstrated partial-subtotal villous atrophy (Marsch 3b) with marked theliocytosis (10 IEL/10 epithelial cells), showing CD3+, CD8- phenotype. PCR revealed monoclonal T-cell gene rearrangement. No preceeding CD was known. The patient was treated with strict diet, steroids, and azathioprine with good partial response. Three control biopsies performed in the past 13 months showed restoration of villous structure and decrease of IEL, but persistence of abnormal IEL and monoclonality.

Conclusions: Our cases are in keeping with reports on RS and proves that some of the cases can be diagnosed by morphological and molecular genetic analysis without previous history of CD. Supported by the Janos Bolyai Research Fellowship of the Hungarian Academy of Sciences.