A case of enteropathy associated T cell lymphoma is reported in a 45 year old woman, presenting with isolated disease in the CNS, three years after diagnosis of coeliac disease. Initial staging showed no evidence of gastrointestinal tract lymphoma. A presumptive diagnosis of T cell primary cerebral lymphoma was made and the patient was treated with combination chemotherapy and craniospinal radiotherapy. The patient relapsed, seven months after treatment, with small bowel lymphoma. The immunophenotype and T cell receptor polymerase chain reaction analysis confirmed the same tumour as in the CNS. Retrospective polymerase chain reaction analysis of intraepithelial lymphocytes in the duodenal biopsy sample, taken at the time of diagnosis of coeliac disease, failed to show evidence of a clonal T cell proliferation.
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