Hope on the Horizon
CHORI Clinical Scientist Confirms Predictive Marker of Acute Chest Syndrome Onset in Pilot Study
In a short report published at the end of the year in the British Journal of Haematology, CHORI clinical scientist Lori Styles, MD, confirmed for the first time that the inflammatory mediator, secretory phospholipase A2 (sPLA2), can be used as a predictive marker in the prevention of acute chest syndrome (ACS) in patients with sickle cell disease (SCD).
"ACS is the number one cause of death in sickle cell disease," says Dr. Styles. "It's like pneumonia, which can be severe in relatively well people, and in sickle cell disease it's even more serious."
As a result, researchers have long been searching for a way to treat or prevent ACS, which normally occurs in sickle cell patients during vaso-occlusive episodes (VOEs). The hallmark of SCD, VOEs are incredibly painful and occur when the blood vessels become clogged due to the sickle shape of the red blood cells.
In this break-through study, Dr. Styles and her colleagues were able to use a predictive marker - an elevation in the levels of sPLA2 - to determine which patients were going to develop ACS, and were then able to prevent ACS onset by pro-actively treating the at-risk patients with blood transfusions.
"It confirms that we can use sPLA2 to predict development, which we've never been able to do. This allows you to prevent ACS rather than simply treat it."
While Dr. Styles' previous research had identified sPLA2 as a potential predictive tool, this is the first study in which that theory was tested and put to use.The results were overwhelming. Of the total number of patients with elevated sPLA2 levels, sixty-three percent of patients randomized to standard care for the treatment of their VOE developed ACS, while none of the patients randomized to transfusion did.
In the mean time, Dr. Styles and her colleagues can celebrate the success of the latest study and the hope that it offers patients with SCD and their families.
"It's really exciting not only because it adds more data to our theory that sPLA2 is a key player," says Dr. Styles, "but also because ACS is a significant problem in sickle cell disease that we haven't ever been able to do anything about - and now we can."Back
Monday, May 16, 2011 11:33 PM