PB1270 - Prevalence of Joint Bleeding in Diverse Inherited Bleeding Disorders; Monocentric data

Background: Major symptom of bleeding is joint bleeding, which can cause serious morbidity and worse quality of life in those who experience it.

Aims: The purpose of this study is to give a thorough analysis of the frequency of joint bleeding in individuals with different types of inherited bleeding disorders.

Methods: In this prospective study inherited bleeding disorder patients diagnosed with hemophilia A, B and von Willebrand disease (vWD) were recruited. This study assessed patient’s demographic details, severity, joints bleeding record, annual bleeding rate (ABR), including quality of life (QOL) and joint scoring (HJHS) which could detect effect of pre and post treatment. Statistical analyses including descriptive analysis was employed to identify significant variations in joint bleed frequencies among the studied patients.

Results: The study cohort included hemophilia (A & B;n=34) among which severe (n=20), moderate (n=10) and mild (n=4) and vWD patients (n=34), vWD type I (n=11), type II (n=5) and type III (n=18) with mean age of 19.7±14.4 years. The total frequency of joint bleeds (65%) varied across the groups with a mean Hemophilia Joint Health Score (HJHS) 10, QOL 52.8% and ABR 60.8%. Children age less than 10 years associated with severe hemophilia were more vulnerable to joint bleeding (80%) with a marked reduction in incidence after treatment (20% = p< 0.05). ABR was 60% in hemophilia and 20% in vWD patients on demand therapy as compared to hemophilia patients on prophylaxis (10%). As compared to Severe hemophilic patients (mean HJHS Score=16), vWD had a higher number of healthy joints (mean HJHS Score=4).

Conclusion(s): This study showed that patients with hemophilia on demand therapy had more ABR and joint bleeding as compared to patients on prophylaxis. Furthermore, patients with vWD had fewer joint bleeds as compared to hemophilia. This highlight’s critical role of prophylaxis in severe bleeding disorder patients.