Abstract

Hematologic primary prohylaxis is the gold standard of treatment in persons with hemophilia (PWH). The goal is to reduce or prevent joint bleeds and subsequent joint degeneration (hemophilic arthropathy). In acute hemarthroses, early treatment with factor (VIII or IX) replacement and rest of the joint (4 to 5 days) are paramount. In patients with inhibitors (antibodies against factor VIII or IX) we can use bypassing agents such as activated prothrombin complex concentrate (aPCC) and recombinant factor VIIa (rFVIIa). The goal is to get the rapid resolution of the joint bleed that must be confirmed by means of ultrasonography (US). This way the risk of long-term complications will be minimized. Ice therapy could help, although its current role in hemophilia remains controversial. Pain killers (paracetamol) may also be needed. Arthrocentesis (joint aspiration) should be performed in very tense and painful joints. The procedure should always be performed under factor coverage and in aseptic conditions. Rehabilitation (physiotherapy) will help recovering the pre-bleeding full range of motion of the joint. In recurrent joint bleeds, radiosynovectomy (RS) and arthroscopic synovectomy (AS) can break the vicious cycle of hemarthrosis-synovitis-hemarthrosis. If joint damage is not avoided, it will compromise the health-related quality of life (HRQoL) of PWH.

Keywords: Hemophilia, hemarthroses, factor replacement, FVIII concentrate, FIX concentrate, aPCC, rFVIIa, radiosynovectomy.

Graphical Abstract