Prophylaxis in adult patients with severe haemophilia A.

Primary prophylaxis is the standard of care for children and adolescents with severe haemophilia; however, its role in adults is less well defined. To establish to which extent prophylaxis is currently being used in adults with severe haemophilia A, we conducted a systematic review of the literature by searching MEDLINE using the terms "prophylaxis", "adult", "severe" and "haemophilia A". Evidence-based guidelines and national studies relating to the use of prophylaxis in adults with severe haemophilia A were identified and reviewed. One published evidence-based guideline and three published studies fulfilled our inclusion criteria. A combined analysis of published data suggests that almost 45% of adult patients with severe haemophilia A are being treated with prophylaxis. Withdrawal of prophylaxis is attempted relatively infrequently; however, when attempted, around 27% of adolescents and adults with severe haemophilia do not experience an increase in bleeding tendency over a specific treatment period. Prophylaxis (primary and secondary) is an important part of haemophilia treatment that should be granted to all patients depending on their clinical presentation. Further studies are needed to better define this treatment strategy in adults.