Coagulation factor replacement in people with haemophilia (PWH) A or B may be given either in response to a bleed [on‐demand (OD) therapy] or regularly to prevent bleeding (prophylactic therapy). Guidelines for prophylactic treatment of children and adults with severe haemophilia A (SHA) were produced by the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) in 2010, summarising the high‐level, evidence‐based studies of prophylaxis in boys and advising on the role of prophylaxis in adults with SHA. This guideline builds on the former, accepting the clear evidence of benefit of prophylaxis in children with SHA. It addresses the optimum use of prophylaxis in children and adults with haemophilia A and B and gives evidence‐based recommendations where appropriate. The guidance will be of value to healthcare professionals, laboratory scientists, patients and those with a responsibility for funding services.

Declaration of Interests

The BSH paid the expenses incurred during the writing of this guidance. None of the authors had conflicts of interest to declare. All authors have made a declaration of interests to the BSH and Task Force Chairs which may be viewed on request.