Risc factors of inhibitor development and quality of life in severe hemophilia A patients

The aim of this study is to investigate the factors that increase the risk of inhibitor development in severe haemophilia A and also to evaluate the quality of life of patients'. A total of 40 patients were included in the study, twnty were inhibitor negative and twenty inhibitor positive. The parameters obtained in both groups were compared. The only parameter that statistically significant was family history of inhibitors (p=0.020). Comparison of FVIII gene mutations of patients', it was found that null mutations had more risk for inhibitory development than non-null mutations, that was not statistically significant but at the borderline (p:0.055). There were no statistically difference between the groups about recombinant FVIII use, prophylaxis, positive family history of haemophilia A and peak treatment days more than 3 days at the diagnosis. Presence of the port catheter was also found to have no effect on the risk of inhibitor development. Patients' bleeding episodes per year and comorbid illnesses were more in inhibitory positive group compared with negatives and these were statistically significant. There were no statistically differerence between groups that absence from work or school, number of radioisotope synovectomy, number of target joints and orthopedic surgery. Statistically comparison of HJHS and Pettersson score of the groups was revealed no difference. The means of EQ5D score, SF-36 total score and SF-36 physical functioning score were lower in inhibitory positive group than negatives and these were statistically significant (p; 0.039, 0.011 ve 0.024). Comparison of other parameters including EQ5D-VAS and SF-36 emotional functioning score showed no statistically difference. In patients with severe hemophilia A, inhibitor development increases the number of bleeding episodes and effects quality of life negatively.