Prevention of bleeding in hemophilia patients with high-titer inhibitors.
Inhibitor development is the most serious adverse event linked to the treatment of hemophilia, as it renders standard hemostatic therapy ineffective. Consequently, inhibitor patients are at increased risk for difficult-to-control bleeding and complications, particularly arthropathy and physical disability. Three randomized clinical trials in patients with inhibitors have demonstrated that compared with on-demand bypassing therapy, prophylaxis with a bypassing agent reduces joint and other types of bleeding and improves health-related quality of life. In hemophilia patients without inhibitors, the initiation of prophylaxis with factor (F) VIII or IX prior to the onset of recurrent hemarthroses can prevent the development of joint disease. Whether this is also true for bypassing agent prophylaxis remains to be determined.
Louisiana Center for Bleeding and Clotting Disorders, Tulane University Medical Center, New Orleans, LA, USA.
Cindy A
CA
Leissinger
Barbara A
BA
Konkle
Sandra V
SV
Antunes
D001779:Blood Coagulation Factors / Q000627:therapeutic use; D062905:Central Venous Catheters / Q000009:adverse effects; D005164:Factor IX / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications*; D006470:Hemorrhage / Q000209:etiology* / Q000517:prevention & control*; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D007239:Infections / Q000209:etiology; D011994:Recombinant Proteins / Q000627:therapeutic use; D013927:Thrombosis / Q000209:etiology
D001779:Blood Coagulation Factors; D006490:Hemostatics; D011994:Recombinant Proteins; C025667:prothrombin complex concentrates; D005169:Factor VIII; D005164:Factor IX; C103587:recombinant FVIIa; D015942:Factor VIIa
activated prothrombin complex concentrate; bypassing agents; factor IX; factor VIII; hemophilia A; hemophilia B; inhibitors; prophylaxis; recombinant activated factor VII