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Case Reports in Pediatrics
Volume 2012 (2012), Article ID 269689, 3 pages
http://dx.doi.org/10.1155/2012/269689
Case Report

Anaplastic Large-Cell Lymphoma in a Child with Type I Diabetes and Unrecognised Coeliac Disease

1Department of Paediatric Gastroenterology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK
2Department of Paediatric Oncology, Alder Hey Children’s NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK
3Department of Paediatric Pathology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool L12 2AP, UK

Received 29 June 2012; Accepted 4 September 2012

Academic Editors: K. Kowal and J. Takaya

Copyright © 2012 Jemima Sharp et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Screening for coeliac disease is recommended for children from certain risk groups, with implications for diagnostic procedures and dietetic management. The risk of a malignant complication in untreated coeliac disease is not considered high in children. We present the case of a girl with type I diabetes who developed weight loss, fatigue, and inguinal lymphadenopathy. Four years before, when she was asymptomatic, a screening coeliac tTG test was positive, but gluten was not eliminated from her diet. Based on clinical examination, a duodenal biopsy, and an inguinal lymph node biopsy were performed, which confirmed both coeliac disease and an anaplastic large-cell lymphoma. HLA-typing demonstrated that she was homozygous for HLA-DQ8, which is associated with higher risk for celiac disease, more severe gluten sensitivity, and diabetes susceptibility. She responded well to chemotherapy and has been in remission for over 4 years. She remains on a gluten-free diet. This is the first case reporting the association of coeliac disease, type I diabetes, and anaplastic large-cell lymphoma in childhood. The case highlights the malignancy risk in a genetically predisposed individual, and the possible role of a perpetuated immunologic response by prolonged gluten exposure.