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Pediatric Inflammatory Bowel Diseases and the Risk of Lymphoma: Should We Revise Our Treatment Strategies?

Cucchiara, Salvatore*; Escher, Johanna C; Hildebrand, Hans; Amil-Dias, Jorge§; Stronati, Laura*; Ruemmele, Frank M||,¶

Journal of Pediatric Gastroenterology & Nutrition: March 2009 - Volume 48 - Issue 3 - p 257–267
doi: 10.1097/MPG.0b013e31818cf555
Invited Review

Inflammatory bowel diseases (IBDs) are lifelong inflammatory gastrointestinal diseases starting in about one third of patients during childhood. Treatment strategies aim to control this chronic inflammatory process. Owing to recent advances in the understanding of IBD, immunosuppressive agents (mainly against TNFα directed) as well as biological drugs are more and more often used. This therapeutic approach clearly improved the clinical condition of the majority of patients with IBD. However, with this more aggressive treatment strategy, safety concerns clearly arise. Recently, the description of a series of a particularly severe form of T cell lymphoma in pediatric and young adult patients with IBD under immunomodulator and biological combination therapy raised the question of the risks of treatment-induced side effects or complications. As reviewed in the present article, there is a slightly increased risk of not only lymphoma development in IBD patients, potentially related to the inflammatory process, but also to the use of immunosuppressive therapies. On the basis of the literature data, we reanalyzed current treatment strategies for children with moderate-to-severe IBD, who are candidates to receive immunomodulator and/or biological agents potentially accelerating the risk of lymphoma development. Comparative clinical studies in IBD are still missing; however, it is prudent to think about adapting immunosuppressive therapies to the inflammatory process of the underlying disorder and if possible to reduce them to monotherapy. Alternative treatment strategies for heavy immunosuppression exist (eg, enteral nutrition in Crohn disease or colectomy in patients with ulcerative colitis) and should be considered whenever appropriate. There is a major need for comparative studies before evidence-based guidelines can be established for safest and best treatment strategies of pediatric patients with IBD.

*Pediatric Gastroenterology Unit, University of Rome La Sapienza, University Hospital Umberto I, Rome, Italy

Department of Pediatric Gastroenterology, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, The Netherlands

Department of Women and Child Health, Astrid Lindgren Children's Hospital, Stockholm, Sweden

§Department of Pediatrics, Pediatric Gastroenterology, Hospital S. João, Porto, Portugal

||AP-HP, Hôpital Necker-Enfants Malades, Department of Pediatrics, Pediatric Gastroenterology

Université Paris-Descartes, Faculté de Médecine Necker, INSERM U793, Paris, France

Received 27 May, 2008

Accepted 4 September, 2008

Address correspondence and reprint requests to Prof Frank M. Ruemmele, MD, PhD, Hôpital Necker Enfants Malades, Pediatric Gastroenterology, 75015 Paris, France (e-mail:

© 2009 Lippincott Williams & Wilkins, Inc.