فهرست نشریات علمی دسترسی آزاد ایران

   Background and Objectives: Developing inhibitors against factors VIII and IX in hemophilia A and B is a major complication, leading to serious clinical consequences and increased healthcare burden. Managing these patients remains a challenge in resource-limited settings. This study evaluated the impact of management strategies on the severity of joint complications in patients with inhibitors.   Methods: This retrospective cohort included 60 patients with severe hemophilia A: 12 with inhibitors receiving bypassing agents on an on-demand regimen and short-term prophylaxis for target joints, and 48 without inhibitors receiving FVIII as long-term prophylaxis. Patients were followed between February 2021 and February 2022 at a major hemophilia comprehensive care center in Iran. Hemarthrosis events and joint damage were assessed through range of motion limitations (LOM) and the Arnold-Hilgartner radiographic grading system. Independent samples t-tests and Mann-Whitney U tests were used for analysis.   Results: Hemarthrosis was more frequent in the inhibitor group (64 vs. 36 events). A higher percentage of inhibitor patients experienced hemarthrosis (75% vs. 39.6%, P = 0.028) and developed target joints (50% vs. 12.5%, P = 0.002). LOM was significantly greater in the inhibitor group (33.3% vs. 8.3%, P = 0.023). Ankle and knee joints were most affected in inhibitor patients, while nearly half of the non-inhibitor group’s hemarthroses occurred in the ankle.   Conclusion: Hemophilia patients with inhibitors on an on-demand regimen develop more joint complications than those without inhibitors receiving long-term prophylaxis. These findings highlight the need for implementing prophylaxis in inhibitor patients to prevent joint damage and improve long-term outcomes.