AI Article Synopsis
- Monogenic deficiencies in interleukin-10 (IL-10) and its receptor (IL-10R) lead to severe inflammatory bowel disease in young children.
- A study of 5 patients with IL-10R deficiencies showed they developed B-cell non-Hodgkin lymphoma between ages 5 and 6, with one case recurring.
- The lymphomas exhibited features similar to diffuse large B-cell lymphomas, linked to the activation of the nuclear factor κB pathway and reduced T-cell presence within the tumors, highlighting the IL-10R pathway's role in cancer development.
Article 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.
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| http://dx.doi.org/10.1182/blood-2013-06-508267 | DOI Listing |
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