Diagnosis and management of haemophilia

In patients with major (head) trauma or major spontaneous bleeding, always give coagulation factor concentrates without delay, before diagnostic imaging or other interventions Prophylactic administration of factor VIII or factor IX concentrate is the standard of care for patients with severe haemophilia in countries where this is economically feasible Avoid drugs that affect haemostasis, such as platelet inhibitors and anticoagulants because they aggravate bleeding symptoms A safety amulet that makes patients with haemophilia easily recognisable may be life saving and is recommended for all patients Advise female relatives of patients with haemophilia to seek genetic counselling because they may be carriers Female carriers may have reduced plasma concentrations of factor VIII or factor IX, similar to those seen in patients with mild haemophilia and may also have bleeding symptoms Sources and selection criteriaWe searched Medline and the Cochrane Database of Systematic Reviews for "haemophilia" and "hemophilia", and we consulted the TRIP database for national guidelines on the management of haemophilia.Finally, we also used our personal reference libraries, giving preference to the highest level of evidence available.32 High KA.Gene therapy for haemophilia: a long and winding road.