NEW research shows that children with an inherited genetic defect in a critical anti-inflammatory pathway have a genetic predisposition to lymphoma.
Results of the study, published in Blood, the Journal of the American Society of Hematology (ASH), reveal an important association between the genetic defect, which causes chronic intestinal inflammation and early onset inflammatory bowel disease, and its role in cancer development in infants and children.
Among the hundreds of signaling pathways in the human immune system that guide the body’s defense against infection, inflammation, and trauma, the interleukin-10 (IL-10) pathway plays a substantial role in regulating and safeguarding the intestinal tract.
In rare cases, a genetic defect can appear in the IL-10 or in one of its receptors (IL-10R1 and IL-10R2) that turns off the pathway’s normal protective function, resulting in the development of very-early-onset inflammatory bowel diseases (VEO-IBD) in children as young as two weeks old.
While chronic intestinal inflammation is a known risk factor for cancer, until this study no formal connection had been made between IL-10 deficiency, VEO-IBD, and the development of certain malignancies.
Researchers began to investigate this potential linkage when five children between 5.5 and 6.5 years of age being monitored for VEO-IBD at the Necker Children’s Hospital in Paris and the Munich Children’s Hospital developed highly proliferative and severe cancer very similar to diffuse large B-cell lymphoma (DLBCL), an extremely rare form of blood cancer in children.
“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,” said lead study author Alain Fischer, MD, PhD, of the Imagine Institute, French National Institute of Health and Medical Research and Assistance Publique – Hôpitaux de Paris in Paris…READ FULL ARTICLE