Study Shows Genetic Predisposition to Lymphoma in Children

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A newly published study indicates that children with an inherited genetic defect in a critical anti-inflammatory pathway have a genetic predisposition to lymphoma.

The findings, reported online in Blood, the journal of the American Society of Hematology (ASH), reveal a link between the genetic defect, which causes chronic intestinal inflammation and very early onset inflammatory bowel disease (VEO-IBD), and its role in cancer development in infants and young children.

The so-called interleukin-10 (IL-10) pathway is a signaling pathway in the human immune system that helps direct the body's response to infection, inflammation and trauma in the intestinal tract. Researchers believe that in certain rare cases, a genetic defect can switch off the immune response function and cause VEO-IBD.

The study

These findings are the first to make a formal link between IL-10 deficiency, VEO-IBD and certain cancers. The study was undertaken after a small handful of children about 6 years of age who were being treated for VEO-IBD at the Necker Children's Hospital in Paris and the Munich Children's Hospital developed otherwise extraordinarily rare cancers. The cancers they developed were very similar to diffuse large B-cell lymphoma (DLBCL).

Said lead study author Alain Fischer, M.D., Ph.D., of the Imagine Institute, French National Institute of Health and Medical Research and Assistance Publique – Hopitaux de Paris in Paris:

When one VEO-IBD patient with an IL-10R deficiency developed diffuse large B-cell lymphoma, we suspected it might be an unfortunate circumstance. However, when the second, third, fourth, and fifth child were diagnosed, it was clear that this was not a chance occurrence ... The confirmed association between the IL-10 pathway and this rare pediatric lymphoma provides a valuable tool to predict cancer risk in children with VEO-IBD so that doctors can take preventive action that may prevent the occurrence or reoccurrence of lymphoma.

This study may offer researchers clues about ways to bolster the protective effects of the IL-10 pathway in an effort to prevent the development of lymphoma, even though the actual mechanism by which this genetic deficiency activates cancer development has not yet been identified.

Source: PR Newswire

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