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Enteropathy-associated T cell lymphoma of the jejunum associated with colonic lymphocytosis with aberrant phenotype
  1. Riccardo Valli1,
  2. Simonetta Piana1,
  3. Fiorella Ilariucci2,
  4. Anna Gazzola3,
  5. Elena Sabattini3
  1. 1Anatomic Pathology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
  2. 2Haematology Unit, Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
  3. 3Haematopathology Unit, S Orsola-Malpighi Hospital, Bologna, Italy
  1. Correspondence to Dr R Valli, Anatomic Pathology Unit, Arcispedale Santa Maria Nuova, Viale Risorgimento 80, 42123 Reggio Emilia, Italy; valli.riccardo{at}

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Enteropathy-associated T cell lymphoma is an uncommon variant of T cell lymphoma and it is often associated with coeliac disease (CD) (80–90% of cases).1 A multifocal increase of intraepithelial lymphocytes (IELs) can occur either in the stomach or in the large bowel in the setting of CD, but, to our knowledge, the co-existence of an enteropathy-associated T cell lymphoma of the small bowel and intraepithelial colonic neoplastic low-grade lesions in a patient without CD has not been described.

A 48-year-old woman came to our attention because of a vague abdominal discomfort. An oesophagogastroduodenoscopy performed to exclude CD turned out to be negative, as did serological testing and HLA DQ2 and DQ8 genotyping. Ten days later, the patient underwent a surgical resection of the jejunum because of intestinal perforation. After diagnosis, staging procedures (bone marrow biopsy, and positron emission tomography and CT scans) were negative for further sites of involvement, and the patient was treated with intensive chemotherapy and autologous stem cell transplantation. Ten months after the diagnosis, the patient was alive, with a poor performance status, but without active disease.

Macroscopically, the surgical specimen consisted of a tract of jejunum with …

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  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.