Journal of Blood Medicine (May 2015)

Acquired hemophilia A: emerging treatment options

  • Janbain M,
  • Leissinger CA,
  • Kruse-Jarres R

Journal volume & issue
Vol. 2015, no. default
pp. 143 – 150

Abstract

Read online

Maissaa Janbain,1 Cindy A Leissinger,1 Rebecca Kruse-Jarres2 1Louisiana Center for Bleeding and Clotting Disorders, Tulane University Medical Center, New Orleans, LA, USA; 2Washington Center for Bleeding Disorders, Blood Works NW, Seattle, WA, USA Abstract: Acquired hemophilia A is a rare autoimmune disorder caused by an autoantibody (inhibitor) to factor VIII (FVIII) that interferes with its coagulant function and predisposes to severe, potentially life-threatening hemorrhage. Disease management focuses on controlling bleeding, primarily with the use of bypassing therapy and recombinant porcine FVIII, and permanently eradicating the autoantibody using various immunosuppressants. Treatment challenges include delayed diagnosis, difficulty achieving hemostasis and durable remissions, and complications associated with the use of hemostatic and immunosuppressive therapy in a primarily older patient population. Keywords: autoantibodies, factor VIII, hemostasis, inhibitors, inhibitor eradication