Two cases of monomorphic epitheliotropic intestinal T-cell lymphoma associated with coeliac disease

Abstract

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive type of intestinal lymphoma which affects individuals without evidence of enteropathy. In this single-centre case series, we describe the first two cases of MEITL in Caucasian patients suffering from histologically-proven coeliac disease (CD). Original medical records were retrieved and anonymised. All biopsy and surgical MEITL specimens were reviewed by three haematopathologists. Two patients aged 63- and 55-year old at CD diagnosis, subsequently developed a MEITL. MEITL always involved the ileum and was multifocal. Both patients died from complications after surgery, including gastrointestinal bleeding, septic shock and multiorgan failure, with a mean survival since MEITL diagnosis of 15.5 ± 16.3 months. In one case, array-CGH revealed a large deletion on chromosome nine between 9p13.1 and 9p24.1, and a recurrent chromosome gain at 9q33-q34. Our cases indicate that a subset of MEITL may arise in Caucasian patients suffering from CD. The clinical, pathological and molecular features of these cases show a partial overlap with enteropathy-associated T-cell lymphoma.

Keywords:

• Enteropathy
• intestine
• lymphoma
• refractory coeliac disease
• villous atrophy

Acknowledgments

We thank Prof. Orsetta Zuffardi for performing array-CGH analysis; Dr. Arturo Bonometti for reviewing all surgical specimens; Dr. Federica Borrelli de Andreis for retrieving data and drafting the manuscript; and Dr. Roberta Riboni for the technical assistance.

Disclosure statement

No potential conflict of interest was reported by the authors.

Author contributions

All the listed authors gave significant contribution to data interpretation, manuscript writing, and final approval for publication. In particular, GRC and MVL designed the study, analysed, interpreted data, and wrote the manuscript. FB, GRC, and ADS, clinically followed-up patients, retrieved and interpreted data, wrote the manuscript, designed the table. ML and MP reviewed and described histopathological specimens, designed the figure. All authors reviewed and approved the manuscript and were involved in major critical revision.