Impact of inhibitors on hemophilia A mortality in the United States

Am J Hematol. 2015 May;90(5):400-5. doi: 10.1002/ajh.23957. Epub 2015 Feb 5.


The previously published mortality studies are limited in hemophilia populations but suggest that there is no increased risk of mortality in factor VIII inhibitor patients. This retrospective study analyzed surveillance data collected on 7,386 males with severe hemophilia A over a 13-year period to assess the association between a current inhibitor and death. During the study period, 432 participants died, among whom 48 were patients with an inhibitor. Clinical characteristics most strongly associated with death were increased number of reported bleeds, signs of liver disease, infection with either HIV or HCV, and the presence of inhibitor. Patients who underwent successful tolerization were not considered inhibitor patients in our analysis. In a multivariable analysis, the odds of death were 70% higher among patients with a current inhibitor compared to those without an inhibitor (P < 0.01). Deaths among patients with inhibitors were much more likely to be attributed to bleeding complications than those among patients without an inhibitor (42 vs. 12%, P < 0.0001). We conclude that males with severe hemophilia A and a current inhibitor are at increased risk of death.

MeSH terms

  • Adolescent
  • Adult
  • Antibodies / blood*
  • Child
  • Cytomegalovirus Infections / blood
  • Cytomegalovirus Infections / complications
  • Cytomegalovirus Infections / drug therapy
  • Cytomegalovirus Infections / mortality*
  • Factor VIII / administration & dosage
  • Factor VIII / antagonists & inhibitors*
  • HIV Infections / blood
  • HIV Infections / complications
  • HIV Infections / drug therapy
  • HIV Infections / mortality*
  • Hemophilia A / blood
  • Hemophilia A / complications
  • Hemophilia A / drug therapy
  • Hemophilia A / mortality*
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Risk Factors
  • Survival Analysis
  • United States


  • Antibodies
  • Factor VIII