Monday, September 25, 2023
This activity is intended for hematologists, radiologists, pediatricians, and other clinicians caring for patients with joint bleeding due to hemophilia.
The goal of this activity is to review the clinical presentation and management of hemarthrosis in patients with hemophilia.
Upon completion of this activity, participants will be able to:
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Hemophilia has been recognized since at least the second century, when it was described in the Talmud,[11] but the management of this coagulopathy is a far newer concept. In 1840, Lane reported the benefits of blood transfusion for controlling hemophilia-related bleeding;[12] in 1923, Feissly demonstrated the superiority of plasma for this purpose[13] and in 1964, Pool discovered cryoprecipitate, ushering in the modern era of hemophilia treatment.[14] The introduction of plasma-derived factor concentrates in the 1970s made it possible for patients to infuse at home at the first sign of bleeding,[15] resulting in a reduction in bleeding-associated morbidity and mortality.[16–18] This impressive forward momentum in treatment was temporarily derailed in the early 1980s, when 70–80% of patients with severe hemophilia became infected with HIV, and virtually all were infected with HCV transmitted from contaminated plasma concentrates.[15,19] In the ensuing decades, the safety of products used for the treatment of hemophilia became an overarching concern of patients, treaters and manufacturers. Today, the availability of recombinant factor concentrates, and plasma-derived products that now undergo two different viral inactivation procedures has minimized or nearly eliminated infectious risk, and inhibitor development is considered the most serious treatment-related complication.
Joint bleeding is the hallmark of hemophilia. In patients with severe FVIII or FIX deficiency, more than 90% of all bleeding episodes occur in the joints, and 80% of these represent hemarthroses of the ankles, knees and elbows.[20] Recurrent bleeding into the same joint, termed a target joint, is when there is recurrent bleeding into the same joint four or more times within a 6-month period of time, results in progressive damage and the development of hemophilic arthropathy, characterized by synovial hypertrophy, cartilage damage, loss of joint space and bony changes.[21,22] Decreased use of a target joint leads to ongoing muscle atrophy, ankylosis, osteoporosis, bone cysts and eventually, crippling arthritis.[21,23,24] The deteriorating health-related quality of life (HRQoL) associated with progressive joint disease[25,26] and the high cost of hospitalization and orthopedic interventions to relieve pain and improve joint function make the prevention of arthropathy a major goal of hemophilia treatment.
