Hemophilia

Hemophilias A and B are hereditary bleeding disorders caused by deficiencies in factor VIII (FVIII) and factor IX (FIX), respectively.  Hemophilia is often treated with replacement factors (ex. recombinant or plasma-derived FVIII or FIX), or bypassing agents (ex. recombinant activated factor VIIa, anti-inhibitor coagulation complex, or recombinant porcine FVIII).  We have shown effects of novel hemostatic agents on fibrin formation, structure, and stability (Wolberg et al, Allen et al, Gray et al).  We are now interested in comparing how replacement and bypassing therapies modify fibrin quality, as well as identifying new, improved drugs to reduce bleeding in hemophilic patients.

Normal and Hemophilic Clots

Confocal micrographs of normal vs. hemophilic clots

Gray LD, Hussey MA, Larson BM, et al. Recombinant factor VIIa analog NN1731 (V158D/E296V/M298Q-FVIIa) enhances fibrin formation, structure and stability in lipidated hemophilic plasma. Thromb Res.2011;128(6):570-576.