Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment

Intestinal lymphomas encompass those lymphomas with a dominant or only localized occurrence in the intestinal tract. Coeliac disease is highly associated with enteropathy-associated T-cell lymphomas (EATLs). Coeliac disease-related lymphomas can appear at nodal or extranodal sites. EATL is often mul...

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Bibliographic Details
Published in:	Scandinavian journal of gastroenterology Vol. 39; no. 241; pp. 78 - 84
Main Authors:	Meijer, J. W. R., Mulder, C. J. J., Goerres, M. G., Boot, H., Schweizer, J. J.
Format:	Journal Article
Language:	English
Published:	England Informa UK Ltd 2004 Taylor & Francis
Subjects:	Aberrant T cells Celiac Disease - complications coeliac disease extraintestinal lymphomas Humans Intestinal Neoplasms - diagnosis Intestinal Neoplasms - etiology Intestinal Neoplasms - therapy Lymphoma, T-Cell - diagnosis Lymphoma, T-Cell - etiology Lymphoma, T-Cell - therapy refractory coeliac disease Risk Factors subcutaneous panniculitis-like T-cell lymphoma T-cell lymphoma ulcerative jejunitis γ/δ lymphoma
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Summary:	Intestinal lymphomas encompass those lymphomas with a dominant or only localized occurrence in the intestinal tract. Coeliac disease is highly associated with enteropathy-associated T-cell lymphomas (EATLs). Coeliac disease-related lymphomas can appear at nodal or extranodal sites. EATL is often multifocal with ulcerative lesions, which explains the high perforation rate at presentation or during chemotherapy. Staging includes ear-nose-throat examination and CT scan of the chest and abdomen. Positron emission tomography (PET) scanning may be valuable. Accurate diagnosis based on endoscopic biopsies is preferable; if necessary, full thickness laparoscopic small-bowel biopsies are mandatory. Refractory coeliac disease (RCD) with aberrant T cells carries a high risk of development of EATLs. There is no satisfactory treatment for EATL, the only possibility of preventing EATL development in RCD being autologous bone marrow transplantation. EATLs can present in 20% of patients as extra-small-bowel T-cell lymphomas; such as subcutaneous panniculitis-like lymphoma, hepatosplenic γ/ lymphoma, nodal as well as sinus, gastric or colon disease and extraintestinal T-cell lymphomas. The majority of EATLs present as large cell lymphoma CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text]; however, they also present as small cell lymphoma CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text]. Sometimes γ/ lymphomas in CD are recognized. Work-up of EATL must include immunohistology, T-cell flow cytometry, T-cell rearrangement and adequate imaging with CT and PET scanning.
ISSN:	0036-5521 0085-5928 1502-7708
DOI:	10.1080/00855920410014605