Modern management of haemophilic arthropathy

Abstract

SummaryCurrently available factor concentrates for treatment of patients with haemophilia are virally inactivated or are made by recombinant technology and their broad use in developed nations has resulted in the dramatic elimination of the treatment‐related viral illnesses that decimated the haemophilia community in the late 20th century. The major morbidity experienced by patients with haemophilia today is joint disease, a result of repeated bleeding episodes into joint spaces. Although administration of factor concentrates to prevent bleeding has been demonstrated to prevent haemophilic joint disease when applied assiduously, repeated bleeding episodes induce synovitis that is irreversible and may progress despite subsequent prophylaxis. Surgical and nuclear medicine interventions are available to reduce the pain of haemophilic arthropathy and to reduce further bleeding episodes. Patients with high titre inhibitors are at great risk for the development of joint disease and present the greatest therapeutic challenges when joint surgery is needed.

Effect of radiosynoviorthesis on the progression of arthropathy and haemarthrosis reduction in haemophilic patients

“…The current literature shows that radiosynovectomy is a safe and efficient therapy . However, during years, the question of which agent is optimal for radio‐nucleotide synovectomy has been a controversial issue . In large joints, such as knee joints, the preferred radionuclide is 90 Y .…”

“…A first‐line treatment for chronic synovitis is radionuclide synoviorthesis, a widely disseminated method providing satisfactory results in adults, children and adolescents . Chemical synoviorthesis with Rifampicin or oxytetracycline chlorhydrate is a good alternative in developing countries where radionuclide is not available, when it is contraindicated or not tolerated by patients …”

Current difficulties and recent advances in bypass therapy for the management of hemophilia with inhibitors: a new and practical formulation of recombinant factor VIIa

“…Similarly, data from a prospective, observational registry comprising 128 bleeding episodes in 15 patients with inhibitors demonstrated numerically lower rebleeding rates among patients treated with rFVIIa within 2 hours (5.2%) versus patients treated more than 2 hours after the first bleeding symptoms appeared (13.7%) 4. By minimizing the extent of bleeding in the joint, prompt treatment may also delay the development of hemophilic arthropathy 6. The improved efficacy of early treatment may also result in reduced bypassing agent consumption by patients.…”

Assessment of Kinect V2 for elbow range of motion estimation in people with haemophilia using an angle correction model

“…Haemarthrosis is the most common morbidity in patients with haemophilia and involves severe disability and reduced health‐related quality of life (HRQoL) . Bleeding occurs into the joints in 70%‐80% of cases, mainly on knees, elbows and ankles .…”