Background: Refractory sprue is defined as primary or secondary failure to respond to a gluten free diet in patients with coeliac disease-like enteropathy and may signify cryptic or overt enteropathy associated T cell lymphoma.
Aims: To study in detail jejunal morphology and immunophenotypes in patients with refractory sprue in the search for features that might be useful to predict prognosis.
Patients: Seven patients are described, representing all such cases identified in our hospital over a 13 year period.
Methods: Biopsy and/or surgical resection specimens were examined by morphology, immunohistochemistry, including enzymatic and immunofluorescent detection, and molecular biology.
Results: All patients had phenotypically abnormal intraepithelial lymphocytes (IELs) that lacked CD8, T cell receptor αβ (or γδ), and/or expressed CD30 in addition to variable expression of the natural killer cell receptor CD94. A monoclonal T cell population was present in six cases, data from the seventh being inconclusive. Three patients had overt lymphoma with CD30+ tumour tissue intervening between intact mucosa that contained neoplastic IELs. Intriguingly, CD30+ IELs were observed both a long way away from, and in direct continuity with, the tumours in these patients. Such CD30+ cells were hardly detected in patients without tumours, two of which are in good health several years after the initial diagnosis.
Conclusions: Our data suggest that abnormal IELs in patients with refractory sprue are phenotypically heterogeneous. CD30 expression by these cells may indicate a worse prognosis, including the occurrence of overt lymphoma.
- refractory sprue
- intraepithelial lymphocytes
- T cell lymphoma
- AGA, antigliadin antibodies
- GFD, gluten free diet
- EATL, enteropathy associated T cell lymphoma
- EBV, Epstein-Barr virus
- EMA, endomysial antibodies
- IELs, intraepithelial lymphocytes
- LCA, leucocyte common antigen
- mAbs, monoclonal antibodies
- PCR, polymerase chain reaction
- TCR, T cell receptor
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