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.
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