Biogen streak lives on with upbeat PhIII results for hemophilia A therapy

Biogen Idec ($BIIB) has secured positive top-line results from a late-stage study of its long-acting clotting factor for a bleeding disorder called hemophilia A. The biotech giant now has positive results from pivotal trials of its two lead candidates in hemophilia, paving the way for regulatory submissions in the U.S. next year and potentially a new commercial franchise for bleeding-disorder treatments.

The company's win comes as other developers of new hemophilia therapies falter. Just this morning Ipsen announced that its hemophilia treatment partner Inspiration declared bankruptcy after the FDA placed a clinical trial for one of their top candidates on hold, and Novo Nordisk ($NVO) nixed development of a long-lasting Factor VIIa product because patients developed inhibitors to the treatment. 

Weston, MA-based Biogen's long-acting Factor VIII candidate appears to have shined. It arrested 98% of bleeding episodes in the study with one of two injections, and the experimental therapy remained active in patients system longer than Baxter International's ($BAX) Factor X treatment Advate, the company said. In the top-line results, Biogen's contender appeared well tolerated and no patients developed resistance or inhibitors to the therapy.

These data follow the positive results from Biogen's study of a long-acting clotting factor for patients with hemophilia B. The therapies both tap Fc fusion technology that extends their half-lives in patients, offering the potential for patients with the rare bleeding disorders to maintain enough clotting factor in their systems with less frequent injections than available therapies. Biogen picked up the technology and candidates in its 2007 buyout of Syntonix Pharmaceuticals in Waltham, MA.

"This represents a new therapeutic area to Biogen," said Dr. Glenn Pierce, the medical chief of Biogen's hemophilia unit, in an interview with FierceBiotech earlier this month. "We've brought in the requisite expertise that is required to be able to develop this area. Although the company is new to hemophilia, the people here are not new to hemophilia. We've been working in this field for a long time."

In the A-LONG trial results revealed today, median annualized bleeding rates were 1.6 in patients on an individualized preventive treatment, 3.6 in those in a weekly prophylaxis arm and 33.6 in those who got injection of the therapy in response to bleeding. The trend shows that individualized dosing was linked with lowered median annualized bleeding rates. In a subset of patients, the terminal half-life of Biogen's Factor VIII was 19 hours compared with 12.4 hours for Baxter's Advate.

Biogen reported that no serious adverse events were linked to the therapy. Outside the perioperative management period, the most common adverse events included inflammation of the upper throat and joints, headache and upper respiratory infections. Safety is paramount for therapies taken for chronic conditions such as hemophilia A.

The biotech powerhouse, which is a top maker of multiple sclerosis drugs, is partnered in Europe with Swedish Orphan Biovitrum (Sobi) on its lead hemophilia therapies. Hemophilia A is more common than hemophilia B, with an estimated 16,000 cases in the U.S. compared with 4,000 for hemophilia B, but both ailments are considered rare bleeding disorders.

- here's the release