Navigation Links
Research shows genetic anti-inflammatory defect predisposes children to lymphoma
Date:10/2/2013

(WASHINGTON, October 2, 2013) 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 online today 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 Hpitaux de Paris in Paris.

To explore the subset and type of the children's lymphomas, investigators performed several analyses to characterize their molecular composition, identify chromosomal abnormalities, and examine their genetic expression profiles. Following these analyses, the research team observed that all five children exhibited sub-types of DLBCL so extraordinarily similar that the similarity could not be atrributed to random occurrence, but rather reflected consequences of the defective IL-10 pathway.

In confirming their discovery, researchers considered that the predisposition to lymphoma in the five children with IL-10 deficiency could have been related to the immunosuppressive therapy that four of them received for VEO-IBD. However, of the 53 children being monitored at the Necker Children's Hospital and the Munich Children's Hospital for VEO-IBD who received the same therapy, the children with IL-10 deficiency were the only ones who developed lymphoma.

While this finding confirms an association between a nonfunctioning IL-10 pathway and lymphoma, the mechanism by which this genetic deficiency activates cancer development has not yet been identified. One hypothesis considers the IL-10 pathway's role in regulating the proliferation of B cells in the body and proposes that an IL-10 deficiency may lead to uncontrolled cell activity and ultimately cancer. Another potential explanation contends that IL-10 deficiency may impair the disease-fighting ability of local T cells, a type of white blood cell.

Given the established protective effects of the IL-10 pathway and the elevated risk of lymphoma observed in these IL-10-deficient children, these findings may lead researchers to develop a more complete understanding of how the IL-10 pathway may be manipulated to aid in cancer prevention.

"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," said Dr. Fischer.


'/>"/>

Contact: Amanda Szabo
aszabo@hematology.org
202-552-4914
American Society of Hematology
Source:Eurekalert

Related medicine news :

1. Researchers launch first-ever phase II safety study of rectal microbicide to prevent HIV
2. CWRU researchers probe brain implant failure and countermeasure
3. Succinic Acid Market is Expected to Reach USD 836.2 Million by 2018: Transparency Market Research
4. Advances in the Obesity Market 2013 Research Now Available at MarketReportsOnline.com
5. Medrio, a Leading EDC Provider for Clinical Research, Announces eClinical Networking and Dinner Seminar
6. Social Media, Internet of Things and the Future of Public Health: Worldwide Industry Latest Market Share, Growth, Size, Trends, Strategy and Forecast Research Report
7. Researchers identify traffic cop for meiosis--with implications for fertility and birth defects
8. Researchers find that drinking fluoridated water gives no additional risks for hip fractures
9. Epigentek to Offer Higher Sensitivity Next Generation Sequencing Research Products
10. New Research Shows Wild Blueberries Have Potential to Improve Heart Health
11. Global Breast Pumps Industry – New Research Available
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/25/2016)... Lewisville, TX (PRWEB) , ... June 25, 2016 , ... ... in the United States, named Dr. Sesan Ogunleye, as the Medical Director of its ... be the facility Medical Director of our new Mesquite location,” said Dr. James M. ...
(Date:6/25/2016)... ... June 25, 2016 , ... On Friday, June 10, Van Mitchell, ... at Work award to iHire in recognition of their exemplary accomplishments in worksite health ... annual Maryland Workplace Health & Wellness Symposium at the BWI Marriott in Linthicum Heights. ...
(Date:6/24/2016)... ... 24, 2016 , ... Those who have experienced traumatic events may suffer from ... avenues, such as drug or alcohol abuse, as a coping mechanism. To avoid this ... coping following a traumatic event. , Trauma sufferers tend to feel a range of ...
(Date:6/24/2016)... ... June 24, 2016 , ... The ... in Dallas that it will receive two significant new grants to support its ... PHA marked its 25th anniversary by recognizing patients, medical professionals and scientists for ...
(Date:6/24/2016)... ... 2016 , ... Comfort Keepers® of San Diego, CA is excited to announce ... program to drive cancer patients to and from their cancer treatments. Comfort Keepers ... of life and ongoing independence. Getting to and from medical treatments is one ...
Breaking Medicine News(10 mins):
(Date:6/24/2016)... , June 24, 2016   Bay ... Rehabilitation Network,s Dean Center for Tick Borne ... Medicine and Rehabilitation, MIT Hacking Medicine, University of ... Innovation, today announced the five finalists of ... Lyme disease.  More than 100 scientists, clinicians, researchers, ...
(Date:6/24/2016)... June 24, 2016 Dehaier Medical Systems Ltd. ... which develops, markets and sells medical devices and wearable ... signed a strategic cooperation agreement with Hongyuan Supply Chain ... Chain") on June 20, 2016, to develop Dehaier,s new ... cooperation agreement, Dehaier will leverage Hongyuan Supply Chain,s sales ...
(Date:6/24/2016)...   Pulmatrix, Inc ., (NASDAQ: PULM ... announced today that it was added to the Russell ... comprehensive set of U.S. and global equity indexes on ... milestone for Pulmatrix," said Chief Executive Officer Robert ... progress in developing drugs for crucial unmet medical needs, ...
Breaking Medicine Technology: