Defective TAFI activation in hemophilia A mice is a major contributor to joint bleeding.

ABSTRACT: Joint bleeds are common in congenital hemophilia but rare in acquired hemophilia A (aHA) for reasons unknown. To identify key mechanisms responsible for joint-specific bleeding in congenital hemophilia, bleeding phenotypes after joint injury and tail transection were compared in aHA wild-type (WT) mice (receiving an anti-factor VIII [FVIII] antibody) and congenital HA (FVIII^-/-) mice. Both aHA and FVIII^-/- mice bled severely after tail transection, but consistent with clinical findings, joint bleeding was notably milder in aHA compared with FVIII^-/- mice. Focus was directed to thrombin-activatable fibrinolysis inhibitor (TAFI) to determine its potentially protective effect on joint bleeding in aHA. Joint bleeding in TAFI^-/- mice with anti-FVIII a

SUBMITTER: Wyseure T 

PROVIDER: S-EPMC6182268 | biostudies-literature | 2018 Oct

REPOSITORIES: biostudies-literature