Comparison of efficacies of low-dose prophylaxis on children and adults with severe hemophilia A in China

LI Zhi-tao; YANG Huan; QIU Shi-qiu; ZHOU Xuan; LIU Yang; SUN Jing

Abstract：Objective To evaluate the efficacies of low -dose prophylaxis on children and adults with severe he-mophilia A.Methods Sixty -four patients with hemophilia A were registered in this retrospective analysis.The adults group and the children group were distinguished by age.In addition, they were divided into prophylaxis group and on -de-mand group according to the treatment regimen.The median annual bleeding rate (ABR), the median annual joint bleed-ing rate (AJBR), the numbers of target joints, the functional independence score in hemophilia (FISH) and the quality of life (QoL) were compared between each groups.Results The ABR of children prophylaxis group (19.7 ±11.1) were significantly lower than that of children on -demand group (54.7 ±20.5) (P =0.000).However, there was no signifi-cant difference in the QoL between these two groups (P =0.928).The ABR of adults prophylaxis group (13.9 ±7.0) were significantly lower than that of adults on -demand group (36.5 ±14.7) (P =0.000).The QoL of adults prophylax-is group were significantly higher than the adults on -demand group (P =0.001).The comparison of efficacies on proph-ylaxis between children and adults showed that it was better in children than in adults in reducing the rate of bleeding.But in improving the quality of life, it was more significant in adult.Conclusion The main effects of prophylaxis therapy in children are to reduce the bleeding of joints and maintain the long -term function of joints.But in adults, whose joints are stable or the damage has been formed, the roles of prophylaxis therapy are in improving the function of damaged joints, self -care ability and quality of life.