The Efficacy of Ultrasound Joint Examination for the Management in Patients with Hemophilia

The regular prophylactic replacement therapy with clotting factors has significantly reduced the arthropathy in patients with hemophilia. Early detection of the signs of joint damage is important to prevent the progressive joint damage. For the early detection, we have introduced the regular ultrasound (US) joint examination in patients with hemophilia from 2014 based on the hemophilia early arthropathy detection with ultrasound (Head-US) scanning protocol. US examination has been performed at the time of annual comprehensive clinic in Hiroshima University Hemophilia Center. Forty-five patients aged 1 to 33 years were enrolled and more than 280 joints were evaluated in this study. US joint examination was performed within 30 minutes without the sedation in all aged children. Abnormal findings, such as cartilage changes, subchondral bone erosion, synovial hypertrophy and/or joint effusion were observed in 8 out of 45 patients. The replacement therapy with clotting factors of all 8 patients aged 13 to 33 years was an insufficient prophylaxis during early childhood. The residual 37 patients aged less than 15 years have relatively followed the prophylactic treatment. The prophylactic regimen has been individually determined based on the trough levels of factor IX concentration in plasma for hemophilia B and pharmacokinetics of factor VIII using MyPKFit^R for hemophilia A patients, respectively. The result suggests the importance of the careful prophylactic replacement therapy considering the factor concentration in plasma.

Four patients aged 4, 5, 18, and 19 years, respectively, suffered from painful and/or swollen joint episodes from 2014 to 2018. Urgent US examination without the use of any sedatives revealed ankle, knee, or hip joint bleeding in each case. According to the replacement therapy for breakthrough bleeding, hemoarthropathy was accessed by consecutive examination of US. These results suggest that US joint examination could be necessary for the early detection of joint damage and for appropriate treatment of hemoarthropathy. Collectively, utilization of US examination would be essential for the management through the life in patients with hemophilia.