| Literature DB >> 24089328 |
Bénédicte Neven1, Emilie Mamessier, Julie Bruneau, Sophie Kaltenbach, Daniel Kotlarz, Felipe Suarez, Julien Masliah-Planchon, Katy Billot, Danielle Canioni, Pierre Frange, Isabelle Radford-Weiss, Vahid Asnafi, Dhaarini Murugan, Christine Bole, Patrick Nitschke, Olivier Goulet, Jean-Laurent Casanova, Stéphane Blanche, Capucine Picard, Olivier Hermine, Frederic Rieux-Laucat, Nicole Brousse, Frederic Davi, Véronique Baud, Christoph Klein, Bertrand Nadel, Frank Ruemmele, Alain Fischer.
Abstract
Monogenic interleukin-10 (IL-10) and IL-10 receptor (IL-10R) deficiencies cause very early onset severe inflammatory bowel disease. Here, we report that 5 patients with an IL-10R1 (n = 1) or IL-10R2 (n = 4) deficiency developed B-cell non-Hodgkin lymphoma between the ages of 5 and 6 years (which was recurrent in 1 patient). These lymphomas had some of the characteristics of diffuse large B-cell lymphomas and contained monoclonal, Epstein-Barr virus-negative germinal center B cells. The tumors displayed a remarkably homogeneous signature, with original activation of the nuclear factor κB pathway and a decrease in intratumor T-cell infiltration. Hence, IL-10R deficiency is associated with a high risk of developing B-cell lymphoma. Our results revealed an unexpected role of the IL-10R pathway in lymphomagenesis.Entities:
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Year: 2013 PMID： 24089328 DOI： 10.1182/blood-2013-06-508267
Source DB: PubMed Journal: Blood ISSN： 0006-4971 Impact factor: 22.113