Bleeding before prophylaxis in severe hemophilia: paradigm shift over two decades

Bleeding before prophylaxis in severe hemophilia: paradigm shift over two decades Prophylaxis for hemophilia is the scheduled infusion of the missing clotting factor with pre-specified dose, with the intention of preventing bleeds and subsequent hemophilic arthropathy. It is the treatment of choice for patients with severe hemophilia A in countries with available resources. 1 Around the year 2000 it was reported that prophylaxis is most efficient when initiated early: before 3 years of age 2 or directly after the first joint bleed. These strategies are reflected in the two most frequently used definitions of primary prophylaxis. The European Pediatric Network for Hemophilia Management (PedNet) first specified primary prophylaxis as starting before two years of age OR before the second joint bleed, 5 and The World Federation of Hemophilia (WFH) as starting before the age of three years AND before the second joint bleed, in the documented absence of osteochondral joint disease.