Alimentary TractAbnormal intestinal intraepithelial lymphocytes in refractory sprue☆,☆☆
Section snippets
Patients with refractory sprue
Six consecutive female adult patients with refractory sprue were studied (group I, Table 1).
Empty Cell Empty Cell Empty Cell Empty Cell Antibodies Empty Cell Empty Cell UPN (sex) Age at onset (yr) Presenting symptoms Associated features AE AG Subsequent response to a GFD Outcome 1070 (F) 46 Weight loss, diarrhea, abdominal pain Sister with GFD-sensitive CD −a + Partial response to GFD for 6 mo only; steroid responsive Alive 5 yr after diagnosis; steroid dependent Ulcerative jejunitis 1398 (F) 46 Diarrhea, malnutrition
Histological results
Sequential duodenal biopsy specimens for standard histology were available for review over a period of 5–12 years for 4 patients and a period of 1–2 years in the 2 other patients. In all patients, total (grade IV) to subtotal (grade III) villous atrophy was present in approximately more than two thirds of the intestinal mucosa examined (Figure 1A), whereas areas with partial villous atrophy were present in the remaining mucosa (Figure 1B).
Discussion
This study provides evidence that patients with unclassified or refractory sprue may be differentiated from celiac disease by the partial or almost complete disappearance of normal subsets of IELs and their replacement by morphologically normal but phenotypically abnormal lymphocytes, which express intracytoplasmic CD3ϵ but not surface CD3ϵ, TCR, CD4, or CD8 and demonstrate restricted rearrangements of the TCRγ gene.
All patients fulfilled the criteria of refractory sprue described by Trier1, 2
Acknowledgements
The authors thank Pr. S. Chaussade (Department of Gastroenterology, Hôpital Cochin, Paris) for providing biopsy specimens from controls; Pr. B. Messing and Pr. C. Matuchansky (Department of Gastroenterology, Hôpital Saint Lazare, Paris) for providing data from 1 patient; Dr. F. Carnot (Department of Pathology, Hôpital Laënnec, Paris) for providing histological specimens; Dr. O. Hermine (Department of Hematology, Hôpital Necker, Paris), Pr. C. Matuchansky (Department of Gastroenterology, Hôpital
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Address requests for reprints to: Christophe Cellier, Ph.D., Service de Gastroentérologie, Hôpital Laënnec, 42 rue de Sèvres, 75007 Paris, France. Fax: (33)1-4439-6812.
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Supported by INSERM, the Assistance Publique des Hôpitaux de Paris (PHRC P960906), and the Fondation de France.