Joint health state in relation to different clinical, laboratory, and radiological variables of hemophilia A patients

Abstract Background Haemophilic (HF) arthropathy is a major complication in haemophilia. Collagen factors are responsible for maintaining the integrity of the vessel wall in the joint. Our goal was to assess the haemophilia joint state and its relation to the clinical, laboratory &radiological data. Methods This cross-sectional study included 50 hemophilia A patients recruited from the Pediatric Hematology / Oncology unit, Children’s Hospital, Ain Shams University,Cairo,Egypt. Assessment of joint status clinicallyand radiologically by X-rays& ultrasound was performed. Results 48% of the studied hemophilia A patients had one, 40 % had two target joints or more. The elbow and knee are the most frequently affected joints (46%,62% respectively).There were no significant differences between inhibitor positive and inhibitor negative patients regarding the number of target joints.However, inhibitor positive patients had significantly lower annual bleeding rate; moreover, none of inhibitor positive patients had surgeries done and none were on celecoxib. There were no significant differences between inhibitor positive hemophilia A patients and inhibitor negative regarding US total score, hem QOL, total HJHS and total FISH score. Patients with 2 target joints had significant higher annual bleeding rate, worse quality of life score, higher total HJHS score compared to those with one or without target joints. Conclusion In our study, we argued the lack of significant difference between inhibitor positive and inhibitor negative Hemophilia A patients as regards the occurrence of hemophilic arthropathy. Nevertheless, patients with ≥ 2 target joints had worse annual bleeding rate, quality of life & total HJHS score.