Genetic Mechanism Of Disease Is Discovered

In the process of figuring out why an anti-cancer drug is effective in treating patients with a rare blood disorder known as hypereosinophilic syndrome, or HES, researchers at Brigham and Women's Hospital (BWH) and the Dana-Farber Cancer Institute have shown that the condition may in fact be a form of cancer.

Their findings appear in the March 27 issue of the New England Journal of Medicine.

HES develops when the level of eosinophils, a white blood cell, rises to abnormal levels in the bloodstream. Sometimes the body produces large amounts of eosinophils as a reaction to allergies or parasites, but why eosinophil levels increase in those patients with HES has remained elusive, until now. Elevated eosinophil levels can lead to heart failure and negatively impact other vital organs, often leading to death.

Imatinib (tradename Gleevec), a drug that has already shown promise in the treatment of chronic myelogenous leukemia, or CML, a form of cancer that involves the overabundance of another kind of white blood cell, was used by Dana-Farber's Daniel DeAngelo, MD, PhD, and Richard Stone, MD, to treat patients with HES. The idea to use this drug derived from both a notion that the mechanism of HES might be similar to CML as well as early reports, ultimately published in the journal Lancet in May 2002, of its utility in HES. The HES patients at DFCI, including one who was very ill, responded extremely well to the drug, although exactly how the drug kept the otherwise aggressive disease at bay was unclear.

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