Home‐delivered ultrasound monitoring for home treatment of haemarthrosis in haemophilia A

The pathogenesis of a haemophilic arthropathy is a process characterized by two main features: haemophilic synovitis and cartilage damage. It has been understood for many years that the iron derived from intra-articular red blood cells plays a central role in the development of both these two main features [1]. The World Federation of Hemophilia (WFH) has published recommendations for the dosage and length of the treatment of haemarthroses in haemophilia [2], but some patients prematurely discontinue their home treatment (HT) when the pain disappears, but acute synovial swelling and blood collection may still persist. This may increase the risk of joint rebleeding (JRB) that can lead to target joints and accelerate the development of haemophilic arthropathy [3]. Since ultrasound imaging is able to objectify the presence of intra-articular blood and synovial swelling, it can be useful to monitor the treatment of haemarthrosis [4,5]. In 2010, we started a home-delivered ultrasound protocol to monitor the HT of haemarthrosis of elbows, knees and ankles applied to all patients with severe haemophilia A (SHA) undergoing secondary prophylaxis. The primary objective of this study was to minimize the incidence of JRB maintaining the length of the HT until the reabsorption of the intraarticular blood and the reduction in the synovial swelling as it was previous to the haemarthrosis. The home-delivered ultrasound programme was offered to all our patients who had SHA and were undergoing secondary prophylaxis (bi-or tri-weekly) at home. The dose and frequency of prophylaxis were monitored to keep the trough levels of factor VIII (FVIII) between 1.5 and 2 IU mL . Only those who accepted the informed consent were finally included in this study. These patients had a clinical and ultrasound joint examination when they had no bleeding episodes to obtain a baseline value. After the appearance of a haemarthrosis on elbows, knees or ankles, the patient would self-administer an initial FVIII dose of 25 IU kg . Subsequently, the patient would inform the hospital, and a physiotherapist (with added qualification in technical imaging) of our unit would go to the patient’s home to perform a clinical and ultrasound analysis within 24 h of the onset of the haemarthrosis. This home-delivered monitoring with ultrasound imaging and physical examinations covered an area of 38 500 km. The diagnosis of haemarthrosis was based on clinical and ultrasound data compared to the basal state. The clinical criteria were the presence or increase in pain, an increased size of the joint perimeter or a reduced range of the useful mobility. The ultrasound criterion was the presence or increase in intra-articular liquid and synovial swelling in relation to the baseline image. Ultrasound examinations were performed using a portable ultrasonography device (Titan SonoSite; Sonosite Inc., Bothell, WA, USA) with a multi-frequency 5–10 MHz linear array-transducer without Power Doppler. The ultrasound examination protocol consisted of the following: (i) For elbows, we examined the posterior olecranon recess, by keeping the joint flexed 90 , placing the transducer in the mid-longitudinal plane using as reference the insertion of the triceps tendon. (ii) For the anterior knee, we examined the patient supine and a knee flexion of approximately 20–30 . The transducer was placed in the mid-longitudinal plane using as a reference the upper pole of the patellar insertion of the quadriceps tendon. Two longitudinal sections (one on each lateral recess) were used with the same reference to the patella with 30 of external inclination while maintaining a knee flexion of 20–30 . (iii) For ankles, we examined 10–20 of plantar flexion. We examined the anterior recess placing the transducer in the midlongitudinal plane between extensor hallux longus tendon and extensor digitorum longus tendon, using as reference the tibial head and talus dome [4,6]. The haemarthroses were treated following the scheme showed in Fig. 1. Correspondence: Jos e A. Aznar, Unidad de Hemostasia y Trombosis, Hospital Universitario y Polit ecnico La Fe, Avda Fernando Abril Martorell 106, 46026 Valencia, Spain. Tel.: +34 961973052; fax: +34 961973052; e-mail: aznar_jan@gva.es