PB0668 - Inhibitor development is associated with decreased quality of life in patients with hemophilia

Background: Inhibitor development leads to a higher rate of disease related complications in patients with hemophilia.

Aims: To assess the Health-related quality of life (HRQoL) in patients with hemophilia with and without inhibitor.

Methods: In this multi-center cross-sectional study, 41 male patients with hemophilia were investigated between May and October 2021in Iran. HRQoL were evaluated by the Short Form-36 (SF36) questionnaire. The relationship of inhibitor and disease severity with Physical Health (PH) and Mental Health (MH) dimensions and the total SF36 was assessed by appropriate tests using SPSS software.

Results: The mean age ± SD of the patients was 36.9 ±13.2 (range: 18-76) years. Approximately half of the patients (51.2%) had severe hemophilia ( < 1% factor activity) and 26.8% were inhibitor positive. Nine patients (22%) had at least one type of blood-borne infections. Patients with severe hemophilia showed significantly lower scores in Physical Function (PF) (P < 0.001), PH dimension (P=0.018), and total SF36 (P=0.031) than patients with mild and moderate hemophilia. Moreover, PF, MH dimension, and total SF36 scores were significantly lower in the inhibitor-positive compared to the none-inhibitor groups (P < 0.001, P=0.045, and P=0.035 respectively) (Table 1). Patients who were complicated by blood-borne infections showed a significantly lower median score in PH dimension than the patients without blood-borne infection (P=0.038). In addition, annual bleeding rate showed significant negative correlation with PH dimension (rs=-0.609, P< 0.001), MH dimension (r=-0.317, P=0.044) and total SF36 (r=-0.455, P=0.003) scores.

Conclusion(s): Reduced HRQoL was observed in hemophilia patients with inhibitor, severe disease and patients with blood-born infections that warrants further attention to these subgroups of patients. Moreover, precise attention should be considered in the prevention and management of bleeding symptoms. On the other hand, improving the social environment of these patients by providing appropriate employment status can improve Physical and Mental health status in these patients.