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refractory sprue

Wednesday 23 November 2005

Adult refractory sprue is a poorly defined disorder.

Synopsis

- severe villous atrophy mimicking coeliac disease but refractory to a strict gluten-free diet
- aberrant intraepithelial lymphoid intestinal population expressing intracytoplasmic CD3 but not surface CD8 (84%)
- clonal intestinal TCRgamma gene rearrangements (76%)
- clonal dissemination to the blood (33%)

See also

- coeliac disease
- enteropathy-associated T-cell lymphoma

References

- Cellier C, Delabesse E, Helmer C, Patey N, Matuchansky C, Jabri B, Macintyre E, Cerf-Bensussan N, Brousse N. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet. 2000 Jul 15;356(9225):203-8. PMID: 10963198