Hemophilic Hemarthroses: Diagnosis and Management

Abstract

Prophylactic factor replacement is the best to reduce or prevent hemarthroses and subsequent joint damage (hemophilic arthropathy). In acute hemarthroses, early treatment with factor (VIII or IX) replacement and rest of the affected limb should allow rapid resolution of the bleeding episode with minimal risk of long-term complications. The affected joint should remain at rest for a short period of time (4–5 days): bed rest for the hip, a sling for the shoulder, and a compressive bandage and plaster splint for the elbow, knee, and ankle. Ice therapy could help to relieve pain and reduce the extent of bleeding, although its current role in hemophilia remains controversial. Analgesics (paracetamol) may also be required, depending on the degree of pain. Arthrocentesis (joint aspiration) should be performed in major hemarthrosis (very tense and painful joints). Joint aspiration should always be done under factor coverage and in aseptic conditions, in order to avoid recurrence or septic arthritis. An early and progressive physical therapy program is then required to recover the full range of movement and the strength of periarticular muscles. In recurrent joint bleeds, radiosynovectomy and arthroscopic synovectomy can effectively control the vicious cycle of hemarthrosis-synovitis-hemarthrosis. Two bypassing agents, a plasma-derived activated prothrombin complex concentrate [aPCC (FEIBA, factor eight inhibitor bypassing agent)] and recombinant factor VIIa (rFVIIa) are currently available for patients with hemophilia who have developed inhibitors.