Featured Research

from universities, journals, and other organizations

Clinical Trial Shows Reduction In Mortality For Children With Severe Langerhans Cell Histiocytosis

Date:
March 1, 2008
Source:
Children's National Medical Center
Summary:
Introducing an increased intensity of chemotherapy in children with severe Langerhans cell histiocytosis can reduce the mortality rate for this disorder by as much as 20 percent when the patient demonstrates a rapid response to such treatment. The LCH clinical trial series, of which this study is the second, are the first-ever randomized clinical trials for the treatment of LCH.

A new international study finds that introducing an increased intensity of chemotherapy in children with severe Langerhans cell histiocytosis (LCH) can reduce the mortality rate for this disorder by as much as 20 percent when the patient demonstrates a rapid response to such treatment.

The study, published in the March 1 edition of Blood, the journal of the American Association of Hematology, is the second in a series of international randomized clinical trials for Langerhans cell histiocytosis coordinated by the Histiocyte Society. The LCH clinical trial series are the first-ever randomized clinical trials for the treatment of LCH.

“The incidence of this particular disorder is so rare, without the cooperation of researchers and participants across the globe, the trial could not have been conducted on a large enough scale to see such positive, and definitive outcomes,” noted senior author Stephan Ladisch, MD, Bosworth Chair for Cancer Biology and Director of the Center for Cancer and Immunology Research at Children’s National Medical Center. “The value of international collaborations such as this is incalculable to the research of so-called ‘orphan diseases’ that receive little government funding support, but still have a significant effect on the lives of children around the world.”

The trial induced a rapid response to the application of chemotherapy—involving prednisone, vinblastine, and for some participants, the addition of etopisode. As a result, the research team observed a statistically significant improved prognosis for children with multi-system LCH. Study leaders observed this significant effect in both study groups of the second clinical trial.

The authors conclude, “There is a strong indication that increasing treatment intensity was associated with higher rapid respond and survival rates. This suggests that therapy intensification…may be essential for successful treatment of high-risk disease.”

The results were based on the findings in 193 pre-identified LCH patients identified as “risk patients”—those with LCH affecting risk organs including the liver, lungs, hematopoeitic system, and spleen, or patients with disease onset younger than 2 years of age. For these high risk groups, mortality rates had been as high as 60 to 70 percent (and are now well below 30 percent).

The results of the first LCH clinical trial (LCHI) were published in 2001 by the Journal of Pediatrics. This new study builds on the findings of LCHI, which focused on the effectiveness of two of the most common treatments of LCH in children.

About Langerhans cell histiocytosis (LCH)

Langerhans cell histiocytosis (LCH) is a rare disorder that primarily affects children, involving clonal proliferation of abnormal cells deriving from bone marrow. Clinically, its manifestations range from isolated bone lesions to multisystemic disease. These cells accumulate in different organs and can result in a variety of symptoms. The cause of LCH is unknown. It is not a known infection or cancer. In severe cases affecting “risk organs” including the liver, lungs and the hematopoeitic system, the disease is fatal. Usually these cases are in very young children.

It is estimated that 9 of every 1,000,000 children under the age of 15 have histiocytosis at various severity levels. Three-quarters of the cases occur before age 10, but the disease also occurs in adults. Because of its rare occurrence, LCH is often referred to as an “orphan” disease.

Journal reference: Blood, 1 March 2008, Vol. 111, No. 5, pp. 2556-2562. Prepublished online as a Blood First Edition Paper on December 18, 2007; DOI 10.1182/blood-2007-08-106211. http://bloodjournal.hematologylibrary.org/cgi/content/abstract/111/5/2556


Story Source:

The above story is based on materials provided by Children's National Medical Center. Note: Materials may be edited for content and length.


Cite This Page:

Children's National Medical Center. "Clinical Trial Shows Reduction In Mortality For Children With Severe Langerhans Cell Histiocytosis." ScienceDaily. ScienceDaily, 1 March 2008. <www.sciencedaily.com/releases/2008/03/080301081638.htm>.
Children's National Medical Center. (2008, March 1). Clinical Trial Shows Reduction In Mortality For Children With Severe Langerhans Cell Histiocytosis. ScienceDaily. Retrieved April 23, 2014 from www.sciencedaily.com/releases/2008/03/080301081638.htm
Children's National Medical Center. "Clinical Trial Shows Reduction In Mortality For Children With Severe Langerhans Cell Histiocytosis." ScienceDaily. www.sciencedaily.com/releases/2008/03/080301081638.htm (accessed April 23, 2014).

Share This



More Health & Medicine News

Wednesday, April 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Big Pharma Braces for M&A Wave

Big Pharma Braces for M&A Wave

Reuters - Business Video Online (Apr. 22, 2014) Big pharma on the move as Novartis boss, Joe Jimenez, tells Reuters about plans to transform his company via an asset exchange with GSK, and Astra Zeneca shares surge on speculation that Pfizer is looking for a takeover. Joanna Partridge reports. Video provided by Reuters
Powered by NewsLook.com
Study Says Most Crime Not Linked To Mental Illness

Study Says Most Crime Not Linked To Mental Illness

Newsy (Apr. 22, 2014) A new study finds most crimes committed by people with mental illness are not caused by symptoms of their illness or disorder. Video provided by Newsy
Powered by NewsLook.com
Hagel Gets Preview of New High-Tech Projects

Hagel Gets Preview of New High-Tech Projects

AP (Apr. 22, 2014) Defense Secretary Chuck Hagel is given hands-on demonstrations Tuesday of some of the newest research from DARPA _ the military's Defense Advanced Research Projects Agency program. (April 22) Video provided by AP
Powered by NewsLook.com
How Smaller Plates And Cutlery Could Make You Feel Fuller

How Smaller Plates And Cutlery Could Make You Feel Fuller

Newsy (Apr. 22, 2014) NBC's "Today" conducted an experiment to see if changing the size of plates and utensils affects the amount individuals eat. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

    Health News

    Environment News

    Technology News



    Save/Print:
    Share:

    Free Subscriptions


    Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

    Get Social & Mobile


    Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

    Have Feedback?


    Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
    Mobile: iPhone Android Web
    Follow: Facebook Twitter Google+
    Subscribe: RSS Feeds Email Newsletters
    Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins